New Avenues for the Treatment of Huntington’s Disease

Abstract

Huntington’s disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the HD gene. The disease is characterized by neurodegeneration, particularly in the striatum and cortex. The first symptoms usually appear in mid-life and include cognitive deficits and motor disturbances that progress over time. Despite being a genetic disorder with a known cause, several mechanisms are thought to contribute to neurodegeneration in HD, and numerous pre-clinical and clinical studies have been conducted and are currently underway to test the efficacy of therapeutic approaches targeting some of these mechanisms with varying degrees of success. Although current clinical trials may lead to the identification or refinement of treatments that are likely to improve the quality of life of those living with HD, major efforts continue to be invested at the pre-clinical level, with numerous studies testing novel approaches that show promise as disease-modifying strategies. This review offers a detailed overview of the currently approved treatment options for HD and the clinical trials for this neurodegenerative disorder that are underway and concludes by discussing potential disease-modifying treatments that have shown promise in pre-clinical studies, including increasing neurotropic support, modulating autophagy, epigenetic and genetic manipulations, and the use of nanocarriers and stem cells.

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    Not in our library10.1016/j.bbrc.2007.05.093

87. 87.

    Altlner Ş., Ardic S., Çebi A.H. Extending the Phenotypic Spectrum of Huntington Disease: Hypothermia. Mol. Syndromol. 2020;11:56-58.

    Not in our library10.1159/000505887

88. 88.

    Terman A., Kurz T., Navratil M., Arriaga E.A., Brunk U.T. Mitochondrial Turnover and aging of long-lived postmitotic cells: The mitochondrial-lysosomal axis theory of aging. Antioxid. Redox Signal. 2010;12:503-535.

    Not in our library10.1089/ars.2009.2598

89. 89.

    Martinez-Vicente M. Autophagy in neurodegenerative diseases: From pathogenic dysfunction to therapeutic modulation. Semin. Cell Dev. Biol. 2015;40:115-126.

    Not in our library10.1016/j.semcdb.2015.03.005

90. 90.

    Weihl C.C. Monitoring Autophagy in the Treatment of Protein Aggregate Diseases: Steps Toward Identifying Autophagic Biomarkers. Neurotherapeutics. 2013;10:383-390.

    Not in our library10.1007/s13311-013-0180-y

91. 91.

    Pircs K., Petri R., Madsen S., Brattås P.L., Vuono R., Ottosson D.R., St-Amour I., Hersbach B.A., Matusiak-Brückner M., Lundh S.H., et al. Huntingtin Aggregation Impairs Autophagy, Leading to Argonaute-2 Accumulation and Global MicroRNA Dysregulation. Cell Rep. 2018;24:1397-1406.

    Not in our library10.1016/j.celrep.2018.07.017

92. 92.

    Ravikumar B., Vacher C., Berger Z., Davies J.E., Luo S., Oroz L.G., Scaravilli F., Easton D.F., Duden R., O’Kane C.J., et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet. 2004;36:585-595.

    Not in our library10.1038/ng1362

93. 93.

    Martinez-Vicente M., Talloczy Z., Wong E., Tang G., Koga H., Kaushik S., De Vries R., Arias E., Harris S., Sulzer D., et al. Cargo recognition failure is responsible for inefficient autophagy in Huntington’s disease. Nat. Neurosci. 2010;13:567-576.

    Not in our library10.1038/nn.2528

94. 94.

    Rui Y.N., Xu Z., Patel B., Chen Z., Chen D., Tito A., David G., Sun Y., Stimming E.F., Bellen H.J., et al. Huntingtin functions as a scaffold for selective macroautophagy. Nat. Cell Biol. 2015;17:262-275.

    Not in our library10.1038/ncb3101

95. 95.

    Koga H., Martinez-Vicente M., Arias E., Kaushik S., Sulzer D., Cuervo A.M. Constitutive upregulation of chaperone-mediated autophagy in Huntington’s disease. J. Neurosci. 2011;31:18492-18505.

    Not in our library10.1523/jneurosci.3219-11.2011

96. 96.

    Cortes C.J., La Spada A.R. The many faces of autophagy dysfunction in Huntington’s disease: From mechanism to therapy. Drug Discov. Today. 2014;19:963-971.

    Not in our library10.1016/j.drudis.2014.02.014

97. 97.

    Kamat P.K., Kalani A., Kyles P., Tyagi S.C., Tyagi N. Autophagy of Mitochondria: A Promising Therapeutic Target for Neurodegenerative Disease. Cell Biochem. Biophys. 2014;70:707-719.

    Not in our library10.1007/s12013-014-0006-5

98. 98.

    Anthony Altar C., Cai N., Bliven T., Juhasz M., Conner J.M., Acheson A.L., Lindsay R.M., Wiegand S.J. Anterograde transport of brain-derived neurotrophic factor and its role in the brain. Nature. 1997;389:856-860.

    Not in our library10.1038/39885

99. 99.

    Baquet Z.C., Gorski J.A., Jones K.R. Early Striatal Dendrite Deficits followed by Neuron Loss with Advanced Age in the Absence of Anterograde Cortical Brain-Derived Neurotrophic Factor. J. Neurosci. 2004;24:4250-4258.

    Not in our library10.1523/jneurosci.3920-03.2004

100. 100.

     Hofer M., Pagliusi S.R., Hohn A., Leibrock J., Barde Y.A. Regional distribution of brain-derived neurotrophic factor mRNA in the adult mouse brain. EMBO J. 1990;9:2459-2464.

     Not in our library10.1002/j.1460-2075.1990.tb07423.x

101. 101.

     Adachi N. New insight in expression, transport, and secretion of brain-derived neurotrophic factor: Implications in brain-related diseases. World J. Biol. Chem. 2014;5:409.

     Not in our library10.4331/wjbc.v5.i4.409

102. 102.

     Chao M.V., Hempstead B.L. p75 and Trk: A two-receptor system. Trends Neurosci. 1995;18:321-326.

     Not in our library10.1016/0166-2236(95)93922-k

103. 103.

     Sugimoto T., Kuroda H., Horii Y., Moritake H., Tanaka T., Hattori S. Signal transduction pathways through TRK-A and TRK-B receptors in human neuroblastoma cells. Jpn. J. Cancer Res. 2001;92:152-160.

     Not in our library10.1111/j.1349-7006.2001.tb01077.x

104. 104.

     Yuen E.C., Mobley W.C. Early BDNF, NT-3, and NT-4 signaling events. Exp. Neurol. 1999;159:297-308.

     Not in our library10.1006/exnr.1999.7148

105. 105.

     Roux P. Neurotrophin signaling through the p75 neurotrophin receptor. Prog. Neurobiol. 2002;67:203-233.

     Not in our library10.1016/s0301-0082(02)00016-3

106. 106.

     Zuccato C. Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington’s Disease. Science. 2001;293:493-498.

     Not in our library10.1126/science.1059581

107. 107.

     Gauthier L.R., Charrin B.C., Borrell-Pagès M., Dompierre J.P., Rangone H., Cordelières F.P., De Mey J., MacDonald M.E., Leßmann V., Humbert S., et al. Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell. 2004;118:127-138.

     Not in our library10.1016/j.cell.2004.06.018

108. 108.

     Yu C., Li C.H., Chen S., Yoo H., Qin X., Park H. Decreased BDNF Release in Cortical Neurons of a Knock-in Mouse Model of Huntington’s Disease. Sci. Rep. 2018;8:1-11.

     Not in our library10.1038/s41598-018-34883-w

109. 109.

     Duan W., Guo Z., Jiang H., Ware M., Li X.J., Mattson M.P. Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice. Proc. Natl. Acad. Sci. USA. 2003;100:2911-2916.

     Not in our library10.1073/pnas.0536856100

110. 110.

     Gines S., Seong I.S., Fossale E., Ivanova E., Trettel F., Gusella J.F., Wheeler V.C., Persichetti F., MacDonald M.E. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington’s disease knock-in mice. Hum. Mol. Genet. 2003;12:497-508.

     Not in our library10.1093/hmg/ddg046

111. 111.

     Giralt A., Carretán O., Lao-Peregrin C., Martín E.D., Alberch J. Conditional BDNF release under pathological conditions improves Huntington’s disease pathology by delaying neuronal dysfunction. Mol. Neurodegener. 2011;6:1-16.

     Not in our library10.1186/1750-1326-6-71

112. 112.

     Jin J., Peng Q., Hou Z., Jiang M., Wang X., Langseth A.J., Tao M., Barker P.B., Mori S., Bergles D.E., et al. Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington’s disease. Hum. Mol. Genet. 2015;24:2508-2527.

     Not in our library10.1093/hmg/ddv016

113. 113.

     Pang T.Y.C., Stam N.C., Nithianantharajah J., Howard M.L., Hannan A.J. Differential effects of voluntary physical exercise on behavioral and brain-derived neurotrophic factor expression deficits in huntington’s disease transgenic mice. Neuroscience. 2006;141:569-584.

     Not in our library10.1016/j.neuroscience.2006.04.013

114. 114.

     Peng Q., Masuda N., Jiang M., Li Q., Zhao M., Ross C.A., Duan W. The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington’s disease mouse model. Exp. Neurol. 2008;210:154-163.

     Not in our library10.1016/j.expneurol.2007.10.015

115. 115.

     Spires T.L., Grote H.E., Garry S., Cordery P.M., Van Dellen A., Blakemore C., Hannan A.J. Dendritic spine pathology and deficits in experience-dependent dendritic plasticity in R6/1 Huntington’s disease transgenic mice. Eur. J. Neurosci. 2004;19:2799-2807.

     Not in our library10.1111/j.0953-816x.2004.03374.x

116. 116.

     Suelves N., Miguez A., López-Benito S., Barriga G.G.D., Giralt A., Alvarez-Periel E., Arévalo J.C., Alberch J., Ginés S., Brito V. Early Downregulation of p75 NTR by Genetic and Pharmacological Approaches Delays the Onset of Motor Deficits and Striatal Dysfunction in Huntington’s Disease Mice. Mol. Neurobiol. 2019;56:935-953.

     Not in our library10.1007/s12035-018-1126-5

117. 117.

     Ciammola A., Sassone J., Cannella M., Calza S., Poletti B., Frati L., Squitieri F., Silani V. Low brain-derived neurotrophic factor (BDNF) levels in serum of Huntington’s disease patients. Am. J. Med. Genet. Part B Neuropsychiatr. Genet. 2007;144:574-577.

     Not in our library10.1002/ajmg.b.30501

118. 118.

     Krzysztoń-Russjan J., Zielonka D., Jackiewicz J., Kuåmirek S., Bubko I., Klimberg A., Marcinkowski J.T., Anuszewska E.L. A study of molecular changes relating to energy metabolism and cellular stress in people with Huntington’s disease: Looking for biomarkers. J. Bioenerg. Biomembr. 2013;45:71-85.

     Not in our library10.1007/s10863-012-9479-3

119. 119.

     Ferrer I., Goutan E., Marín C., Rey M.J., Ribalta T. Brain-derived neurotrophic factor in Huntington disease. Brain Res. 2000;866:257-261.

     Not in our library10.1016/s0006-8993(00)02237-x

120. 120.

     Seo H., Sonntag K.C., Isacson O. Generalized brain and skin proteasome inhibition in Huntington’s disease. Ann. Neurol. 2004;56:319-328.

     Not in our library10.1002/ana.20207

121. 121.

     Zuccato C., Marullo M., Conforti P., MacDonald M.E., Tartari M., Cattaneo E. Systematic assessment of BDNF and its receptor levels in human cortices affected by Huntington’s disease. Brain Pathol. 2008;18:225-238.

     Not in our library10.1111/j.1750-3639.2007.00111.x

122. 122.

     Squitieri F., Orobello S., Cannella M., Martino T., Romanelli P., Giovacchini G., Frati L., Mansi L., Ciarmiello A. Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins. Eur. J. Nucl. Med. Mol. Imaging. 2009;36:1113-1120.

     Not in our library10.1007/s00259-009-1103-3

123. 123.

     Pan W., Banks W.A., Kastin A.J. Permeability of the blood-brain barrier to neurotrophins. Brain Res. 1998;788:87-94.

     Not in our library10.1016/s0006-8993(97)01525-4

124. 124.

     Pan W., Banks W.A., Fasold M.B., Bluth J., Kastin A.J. Transport of brain-derived neurotrophic factor across the blood-brain barrier. Neuropharmacology. 1998;37:1553-1561.

     Not in our library10.1016/s0028-3908(98)00141-5

125. 125.

     Karege F., Schwald M., Cisse M. Postnatal developmental profile of brain-derived neurotrophic factor in rat brain and platelets. Neurosci. Lett. 2002;328:261-264.

     Not in our library10.1016/s0304-3940(02)00529-3

126. 126.

     Zuccato C., Cattaneo E. Role of brain-derived neurotrophic factor in Huntington’s disease. Prog. Neurobiol. 2007;81:294-330.

     Not in our library10.1016/j.pneurobio.2007.01.003

127. 127.

     Menalled L.B., Chesselet M.-F. Mouse models of Huntington’s disease. Trends Pharmacol. Sci. 2002;23:32-39.

     Not in our library10.1016/s0165-6147(00)01884-8

128. 128.

     Hickey M.A., Chesselet M.F. The use of transgenic and knock-in mice to study Huntington’s disease. Cytogenet. Genome Res. 2003;100:276-286.

     Not in our library10.1159/000072863

129. 129.

     Levine M.S., Cepeda C., Hickey M.A., Fleming S.M., Chesselet M.F. Genetic mouse models of Huntington’s and Parkinson’s diseases: Illuminating but imperfect. Trends Neurosci. 2004;27:691-697.

     Not in our library10.1016/j.tins.2004.08.008

130. 130.

     Gil J.M., Rego A.C. The R6 lines of transgenic mice: A model for screening new therapies for Huntington’s disease. Brain Res. Rev. 2009;59:410-431.

     Not in our library10.1016/j.brainresrev.2008.12.001

131. 131.

     Gil-Mohapel J.M. Screening of therapeutic strategies for Huntington’s disease in YAC128 transgenic mice. CNS Neurosci. Ther. 2012;18:77-86.

     Not in our library10.1111/j.1755-5949.2011.00246.x

132. 132.

     Farshim P.P., Bates G.P. Mouse Models of Huntington’s Disease. Methods in Molecular Biology. 2018;Volume 1780:97-120.

     Not in our library10.1007/978-1-4939-7825-0_6

133. 133.

     Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S.W., et al. Exon I of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell. 1996;87:493-506.

     Not in our library10.1016/s0092-8674(00)81369-0

134. 134.

     Lione L.A., Carter R.J., Hunt M.J., Bates G.P., Morton A.J., Dunnett S.B. Selective discrimination learning impairments in mice expressing the human Huntington’s disease mutation. J. Neurosci. 1999;19:10428-10437.

     Not in our library10.1523/jneurosci.19-23-10428.1999

135. 135.

     Carter R.J., Lione L.A., Humby T., Mangiarini L., Mahal A., Bates G.P., Dunnett S.B., Morton A.J. Characterization of Progressive Motor Deficits in Mice Transgenic for the Human Huntington’s Disease Mutation. J. Neurosci. 1999;19:3248-3257.

     Not in our library10.1523/jneurosci.19-08-03248.1999

136. 136.

     Davies S.W., Turmaine M., Cozens B.A., Di Figlia M., Sharp A.H., Ross C.A., Scherzinger E., Wanker E.E., Mangiarini L., Bates G.P. Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation. Cell. 1997;90:537-548.

     Not in our library10.1016/s0092-8674(00)80513-9

137. 137.

     Dodds L., Chen J., Berggren K., Fox J. Characterization of Striatal Neuronal Loss and Atrophy in the R6/2 Mouse Model of Huntington’s Disease. PLoS Curr. 2014.

     Not in our library10.1371/currents.hd.48727b68b39b82d5fe350f753984bcf9

138. 138.

     Stack E.C., Kubilus J.K., Smith K., Cormier K., Del Signore S.J., Guelin E., Ryu H., Hersch S.M., Ferrante R.J. Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington’s disease transgenic mice. J. Comp. Neurol. 2005;490:354-370.

     Not in our library10.1002/cne.20680

139. 139.

     Schilling G., Becher M.W., Sharp A.H., Jinnah H.A., Duan K., Kotzuk J.A., Slunt H.H., Ratovitski T., Cooper J.K., Jenkins N.A., et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum. Mol. Genet. 1999;8:397-407.

     Not in our library10.1093/hmg/8.3.397

140. 140.

     Cheng Y., Peng Q., Hou Z., Aggarwal M., Zhang J., Mori S., Ross C.A., Duan W. Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington’s disease mouse model. Neuroimage. 2011;56:1027-1034.

     Not in our library10.1016/j.neuroimage.2011.02.022

141. 141.

     Yu Z.X., Li S.H., Evans J., Pillarisetti A., Li H., Li X.J. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington’s disease. J. Neurosci. 2003;23:2193-2202.

     Not in our library10.1523/jneurosci.23-06-02193.2003

142. 142.

     Southwell A.L., Ko J., Patterson P.H. Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington’s disease. J. Neurosci. 2009;29:13589-13602.

     Not in our library10.1523/jneurosci.4286-09.2009

143. 143.

     Slow E.J., van Raamsdonk J., Rogers D., Coleman S.H., Graham R.K., Deng Y., Oh R., Bissada N., Hossain S.M., Yang Y.Z., et al. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 2003;12:1555-1567.

     Not in our library10.1093/hmg/ddg169

144. 144.

     Pouladi M.A., Graham R.K., Karasinska J.M., Xie Y., Santos R.D., Petersn Â., Hayden M.R. Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin. Brain. 2009;132:919-932.

     Not in our library10.1093/brain/awp006

145. 145.

     Brooks S., Higgs G., Janghra N., Jones L., Dunnett S.B. Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6J) Huntington’s disease transgenic mice. Brain Res. Bull. 2012;88:113-120.

     Not in our library10.1016/j.brainresbull.2010.05.005

146. 146.

     Van Raamsdonk J.M., Murphy Z., Slow E.J., Leavitt B.R., Hayden M.R. Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 2005;14:3823-3835.

     Not in our library10.1093/hmg/ddi407

147. 147.

     Bayram-Weston Z., Jones L., Dunnett S.B., Brooks S.P. Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington’s disease transgenic mice. Brain Res. Bull. 2012;88:137-147.

     Not in our library10.1016/j.brainresbull.2011.05.005

148. 148.

     Pouladi M.A., Stanek L.M., Xie Y., Franciosi S., Southwell A.L., Deng Y., Butland S., Zhang W., Cheng S.H., Shihabuddin L.S., et al. Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice. Hum. Mol. Genet. 2012;21:2219-2232.

     Not in our library10.1093/hmg/dds037

149. 149.

     Gray M., Shirasaki D.I., Cepeda C., André V.M., Wilburn B., Lu X.H., Tao J., Yamazaki I., Li S.H., Sun Y.E., et al. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J. Neurosci. 2008;28:6182-6195.

     Not in our library10.1523/jneurosci.0857-08.2008

150. 150.

     Spampanato J., Gu X., Yang X.W., Mody I. Progressive synaptic pathology of motor cortical neurons in a BAC transgenic mouse model of Huntington’s disease. Neuroscience. 2008;157:606-620.

     Not in our library10.1016/j.neuroscience.2008.09.020

151. 151.

     Southwell A.L., Skotte N.H., Villanueva E.B., Østergaard M.E., Gu X., Kordasiewicz H.B., Kay C., Cheung D., Xie Y., Waltl S., et al. A novel humanizedmouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles. Hum. Mol. Genet. 2017;26:1115-1132.

     Not in our library10.1093/hmg/ddx021

152. 152.

     Southwell A.L., Warby S.C., Carroll J.B., Doty C.N., Skotte N.H., Zhang W., Villanueva E.B., Kovalik V., Xie Y., Pouladi M.A., et al. A fully humanized transgenic mouse model of Huntington disease. Hum. Mol. Genet. 2013;22:18-34.

     Not in our library10.1093/hmg/dds397

153. 153.

     Menalled L.B. Knock-in mouse models of Huntington’s disease. NeuroRx. 2005;2:465-470.

     Not in our library10.1602/neurorx.2.3.465

154. 154.

     Lin C.H., Tallaksen-Greene S., Chien W.M., Cearley J.A., Jackson W.S., Crouse A.B., Ren S., Li X.J., Albin R.L., Detloff P.J. Neurological abnormalities in a knock-in mouse model of Huntington’s disease. Hum. Mol. Genet. 2001;10:137-144.

     Not in our library10.1093/hmg/10.2.137

155. 155.
     Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175 · 2012

     No notice
156. 156.

     Peng Q., Wu B., Jiang M., Jin J., Hou Z., Zheng J., Zhang J., Duan W. Characterization of behavioral, neuropathological, brain metabolic and key molecular changes in zQ175 knock-in mouse model of huntington’s disease. PLoS ONE. 2016;11:e0148839.

     Not in our library10.1371/journal.pone.0148839

157. 157.

     Kumar A., Kumar V., Singh K., Kumar S., Kim Y., Lee Y.-M., Kim J.-J. Therapeutic Advances for Huntington’s Disease. Brain Sci. 2020;10:43.

     Not in our library10.3390/brainsci10010043

158. 158.

     Stahl C.M., Feigin A. Medical, Surgical, and Genetic Treatment of Huntington Disease. Neurol. Clin. 2020;38:367-378.

     Not in our library10.1016/j.ncl.2020.01.010

159. 159.

     Pan L., Feigin A. Huntington’s Disease: New Frontiers in Therapeutics. Curr. Neurol. Neurosci. Rep. 2021;21:10.

     Not in our library10.1007/s11910-021-01093-3

160. 160.

     Shannon K.M. Recent Advances in the Treatment of Huntington’s Disease: Targeting DNA and RNA. CNS Drugs. 2020;34:219-228.

     Not in our library10.1007/s40263-019-00695-3

161. 161.

     Beatriz M., Lopes C., Ribeiro A.C.S., Rego A.C.C. Revisiting cell and gene therapies in Huntington’s disease. J. Neurosci. Res. 2021;1-19.

     Not in our library10.1002/jnr.24845

162. 162.

     Wyant K.J., Ridder A.J., Dayalu P. Huntington’s Disease—Update on Treatments. Curr. Neurol. Neurosci. Rep. 2017;17:33.

     Not in our library10.1007/s11910-017-0739-9

163. 163.

     Potkin K.T., Potkin S.G. New directions in therapeutics for Huntington disease. Future Neurol. 2018;13:101-121.

     Not in our library10.2217/fnl-2017-0035

164. 164.

     Armstrong M.J., Miyasaki J.M. Evidence-based guideline: Pharmacologic treatment of chorea in Huntington disease: Report of the guideline development subcommittee of the American academy of neurology. Neurology. 2012;79:597-603.

     Not in our library10.1212/wnl.0b013e318263c443

165. 165.

     Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: A randomized controlled trial. Neurology. 2006;66:366-372.

     Not in our library10.1212/01.wnl.0000198586.85250.13

166. 166.

     Frank S., Ondo W., Fahn S., Hunter C., Oakes D., Plumb S., Marshall F., Shoulson I., Eberly S., Walker F., et al. A study of chorea after tetrabenazine withdrawal in patients with Huntington disease. Clin. Neuropharmacol. 2008;31:127-133.

     Not in our library10.1097/wnf.0b013e3180ca77ea

167. 167.

     Paulsen J.S., Hoth K.F., Nehl C., Stierman L. Critical periods of suicide risk in Huntington’s disease. Am. J. Psychiatry. 2005;162:725-731.

     Not in our library10.1176/appi.ajp.162.4.725

168. 168.

     Huntington Study Group Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease. JAMA. 2016;316:40.

     Not in our library10.1001/jama.2016.8655

169. 169.

     Burgunder J.-M., Guttman M., Perlman S., Goodman N., van Kammen D.P., Goodman L. An International Survey-based Algorithm for the Pharmacologic Treatment of Chorea in Huntington’s Disease. PLoS Curr. 2011;3:RRN1260.

     Not in our library10.1371/currents.rrn1260

170. 170.

     Coppen E.M., Roos R.A.C. Current Pharmacological Approaches to Reduce Chorea in Huntington’s Disease. Drugs. 2017;77:29-46.

     Not in our library10.1007/s40265-016-0670-4

171. 171.

     Dallocchio C., Buffa C., Tinelli C., Mazzarello P. Effectiveness of Risperidone in Huntington Chorea Patients. J. Clin. Psychopharmacol. 1999;19:101-103.

     Not in our library10.1097/00004714-199902000-00020

172. 172.

     Bonelli R., Wenning G. Pharmacological Management of Huntingtons Disease: An Evidence- Based Review. Curr. Pharm. Des. 2006;12:2701-2720.

     Not in our library10.2174/138161206777698693

173. 173.

     Bonelli R.M., Mahnert F.A., Niederwieser G. Olanzapine for Huntington’s Disease: An Open Label Study. Clin. Neuropharmacol. 2002;25:263-265.

     Not in our library10.1097/00002826-200209000-00007

174. 174.

     Ciammola A., Sassone J., Colciago C., Mencacci N.E., Poletti B., Ciarmiello A., Squitieri F., Silani V. Aripiprazole in the treatment of Huntington’s disease: A case series. Neuropsychiatr. Dis. Treat. 2009;5:1-4.

     Not in our library

175. 175.

     Brusa L., Orlacchio A., Moschella V., Iani C., Bernardi G., Mercuri N.B. Treatment of the symptoms of Huntington’s disease: Preliminary results comparing aripiprazole and tetrabenazine. Mov. Disord. 2009;24:126-129.

     Not in our library10.1002/mds.22376

176. 176.

     Madhusoodanan S., Brenner R. Use of Risperidone in Psychosis Associated With Huntington’s Disease. Am. J. Geriatr. Psychiatry. 1998;6:347-349.

     Not in our library10.1097/00019442-199800640-00013

177. 177.

     Erdemoglu A.K., Boratav C. Risperidone in chorea and psychosis of Huntington’s disease. Eur. J. Neurol. 2002;9:182-183.

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178. 178.

     Cankurtaran E.S., Ozalp E., Soygur H., Cakir A. Clinical experience with risperidone and memantine in the treatment of Huntington’s disease. J. Natl. Med. Assoc. 2006;98:1353-1355.

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179. 179.

     Reveley M.A., Dursun S.M., Andrews H. A comparative trial use of sulpiride and risperidone in Huntington’s disease: A pilot study. J. Psychopharmacol. 1996;10:162-165.

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180. 180.

     Lucetti C., Gambaccini G., Bernardini S., Dell’Agnello G., Petrozzi L., Rossi G., Bonuccelli U. Amantadine in Huntington’s disease: Open-label video-blinded study. Neurol. Sci. 2002;23:s83-s84.

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181. 181.

     Lucetti C., Del Dotto P., Gambaccini G., Dell’ Agnello G., Bernardini S., Rossi G., Murri L., Bonuccelli U. IV amantadine improves chorea in Huntington’s disease: An acute randomized, controlled study. Neurology. 2003;60:1995-1997.

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182. 182.

     Metman L.V., Morris M.J., Farmer C., Gillespie M., Mosby K., Wuu J., Chase T.N. Huntington’s disease: A randomized, controlled trial using the NMDA-antagonist amantadine. Neurology. 2002;59:694-699.

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183. 183.

     O’Suilleabhain P., Dewey R.B. A randomized trial of amantadine in Huntington disease. Arch. Neurol. 2003;60:996-998.

     Not in our library10.1001/archneur.60.7.996

184. 184.

     Huntington Study Group. Dosage effects of riluzole in Huntington’s disease: A multicenter placebo-controlled study. Neurology. 2003;61:1551-1556.

     Not in our library10.1212/01.wnl.0000096019.71649.2b

185. 185.

     Landwehrmeyer G.B., Dubois B., De Yébenes J.G., Kremer B., Gaus W., Kraus P.H., Przuntek H., Dib M., Doble A., Fischer W., et al. Riluzole in Huntington’s disease: A 3-year, randomized controlled study. Ann. Neurol. 2007;62:262-272.

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186. 186.

     Racette B.A., Perlmutter J.S. Levodopa responsive parkinsonism in an adult with Huntington’s disease. J. Neurol. Neurosurg. Psychiatry. 1998;65:577-579.

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187. 187.

     Glidden A.M., Luebbe E.A., Elson M.J., Goldenthal S.B., Snyder C.W., Zizzi C.E., Dorsey E.R., Heatwole C.R. Patient-reported impact of symptoms in Huntington disease: PRISM-HD. Neurology. 2020;94:e2045-e2053.

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188. 188.

     Paulsen J.S., Butters N., Sadek J.R., Johnson S.A., Salmon D.P., Swerdlow N.R., Swenson M.R. Distinct cognitive profiles of cortical and subcortical dementia in advanced illness. Neurology. 1995;45:951-956.

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189. 189.

     Hoth K.F., Paulsen J.S., Moser D.J., Tranel D., Clark L.A., Bechara A. Patients with Huntington’s disease have impaired awareness of cognitive, emotional, and functional abilities. J. Clin. Exp. Neuropsychol. 2007;29:365-376.

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190. 190.

     Fernandez H.H., Friedman J.H., Grace J., Beason-Hazen S. Donepezil for Huntington’s disease. Mov. Disord. 2000;15:173-176.

     Not in our library10.1002/1531-8257(200001)15:1<173::aid-mds1032>3.0.co;2-t

191. 191.

     Rot U., Kobal J., Sever A., Pirtosek Z., Mesec A. Rivastigmine in the treatment of Huntington’s disease. Eur. J. Neurol. 2002;9:689-690.

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192. 192.

     De Tommaso M., Specchio N., Sciruicchio V., Difruscolo O., Specchio L.M. Effects of rivastigmine on motor and cognitive impairment in Huntington’s disease. Mov. Disord. 2004;19:1516-1518.

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193. 193.

     Petrikis P., Andreou C., Piachas A., Bozikas V.P., Karavatos A. Treatment of Huntington’s disease with galantamine. Int. Clin. Psychopharmacol. 2004;19:49-50.

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194. 194.

     Ondo W.G., Mejia N.I., Hunter C.B. A pilot study of the clinical efficacy and safety of memantine for Huntington’s disease. Parkinsonism Relat. Disord. 2007;13:453-454.

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195. 195.

     Beister A., Kraus P., Kuhn W., Dose M., Weindl A., Gerlach M. The N-methyl-D-aspartate antagonist memantine retards progression of Huntington’s disease. J. Neural Transm. Suppl. 2004;117-122.

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196. 196.

     Van Duijn E., Kingma E.M., Van Der Mast R.C. Psychopathology in verified Huntington’s disease gene carriers. J. Neuropsychiatry Clin. Neurosci. 2007;19:441-448.

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197. 197.

     Van Duijn E., Craufurd D., Hubers A.A.M., Giltay E.J., Bonelli R., Rickards H., Anderson K.E., Van Walsem M.R., Van Der Mast R.C., Orth M., et al. Neuropsychiatric symptoms in a European Huntington’s disease cohort (REGISTRY). J. Neurol. Neurosurg. Psychiatry. 2014;85:1411-1418.

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198. 198.

     Paulsen J.S., Ready R.E., Hamilton J.M., Mega M.S., Cummings J.L. Neuropsychiatric aspects of Huntington’s disease. J. Neurol. Neurosurg. Psychiatry. 2001;71:310-314.

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199. 199.

     Moulton C.D., Hopkins C.W.P., Bevan-Jones W.R. Systematic review of pharmacological treatments for depressive symptoms in Huntington’s disease. Mov. Disord. 2014;29:1556-1561.

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200. 200.

     Rosenblatt A., Ranen N.G., Nance M.A., Paulsen J.S. A Physician’s Guide to the Management of Huntington Disease. 1999.

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201. 201.

     Paleacu D., Anca M., Giladi N. Olanzapine in Huntington’s disease. Acta Neurol. Scand. 2002;105:441-444.

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202. 202.

     Squitieri F., Cannella M., Porcellini A., Brusa L., Simonelli M., Ruggieri S. Short-term effects of olanzapine in Huntington disease. Neuropsychiatry Neuropsychol. Behav. Neurol. 2001;14:69-72.

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203. 203.

     Duff K., Beglinger L.J., O’Rourke M.E., Nopoulos P., Paulson H.L., Paulsen J.S. Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington’s disease. Ann. Clin. Psychiatry. 2008;20:1-3.

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204. 204.

     Sajatouic M., Verbanac P., Ramirez L.F., Meltzer H.Y. Clozapine treatment of psychiatric symptoms resistant to neuroleptic treatment in patients with Huntington’s chorea. Neurology. 1991;41:156.

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205. 205.

     Groves M., van Duijn E., Anderson K., Craufurd D., Edmondson M.C., Goodman N., van Kammen D.P., Goodman L. An International Survey-based Algorithm for the Pharmacologic Treatment of Irritability in Huntington’s Disease. PLoS Curr. 2011;3:RRN1259.

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206. 206.

     Anderson K., Craufurd D., Edmondson M.C., Goodman N., Groves M., van Duijn E., van Kammen D.P., Goodman L.V. An International survey-based algorithm for the pharmacologic treatment of obsessive-compulsive behaviors in Huntington’s disease. PLoS Curr. 2011;3:RRN1261.

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207. 207.

     Oosterloo M., Craufurd D., Nijsten H., Van Duijn E. Obsessive-Compulsive and Perseverative Behaviors in Huntington’s Disease. J. Huntingtons. Dis. 2019;8:1-7.

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208. 208.

     Seitz D.P., Millson R.C. Quetiapine in the Management of Psychosis Secondary to Huntington’s Disease: A Case Report. Can. J. Psychiatry. 2004;49:413.

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209. 209.

     Alpay M., Koroshetz W.J. Quetiapine in the treatment of behavioral disturbances in patients with Huntington’s disease. Psychosomatics. 2006;47:70-72.

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210. 210.

     Uhlyar S., Rey J.A. Valbenazine (Ingrezza): The first FDA-approved treatment for tardive dyskinesia. Pharm. Ther. 2018;43:328-331.

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211. 211.

     Leysen J.E., Janssen P.M.F., Megens A.A.H.P., Schotte A. Risperidone: A novel antipsychotic with balanced serotonin-dopamine antagonism, receptor occupancy profile, and pharmacologic activity. J. Clin. Psychiatry. 1994;55:5-12.

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212. 212.

     Taylor C.P., Traynelis S.F., Siffert J., Pope L.E., Matsumoto R.R. Pharmacology of dextromethorphan: Relevance to dextromethorphan/quinidine (Nuedexta®) clinical use. Pharmacol. Ther. 2016;164:170-182.

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213. 213.

     Amin L., Le N., Mercer R.C.C., Germann U., Alam J., Harris D.A. Role of P38α MAP kinase in amyloid-β derived diffusible ligand (ADDL) induced dendritic spine loss in hippocampal neurons. Alzheimers Dement. 2019;15:P1507.

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214. 214.

     Jiang Y., Stavrides P., Darji S., Yang D.-S., Bleiwas C., Smiley J., Germann U., Alam J., Nixon R. Effects of P38α MAP kinase inhibition on the neurodegenerative phenotype of the TS2 Down Syndrome mouse model. Alzheimers Dement. 2019;15:P1597.

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215. 215.

     Valero T. Editorial (Thematic Issue: Mitochondrial Biogenesis: Pharmacological Approaches). Curr. Pharm. Des. 2014;20:5507-5509.

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216. 216.

     Adanyeguh I.M., Rinaldi D., Henry P.G., Caillet S., Valabregue R., Durr A., Mochel F. Triheptanoin improves brain energy metabolism in patients with Huntington disease. Neurology. 2015;84:490-495.

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217. 217.

     Vázquez-Manrique R.P., Farina F., Cambon K., Dolores Sequedo M., Parker A.J., Millán J.M., Weiss A., Déglon N., Neri C. AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of huntington’s disease. Hum. Mol. Genet. 2015;25:1043-1058.

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218. 218.

     Sanchis A., García-Gimeno M.A., Cañada-Martínez A.J., Sequedo M.D., Millán J.M., Sanz P., Vázquez-Manrique R.P. Metformin treatment reduces motor and neuropsychiatric phenotypes in the zQ175 mouse model of Huntington disease. Exp. Mol. Med. 2019;51:1-16.

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219. 219.

     Ma T.C., Buescher J.L., Oatis B., Funk J.A., Nash A.J., Carrier R.L., Hoyt K.R. Metformin therapy in a transgenic mouse model of Huntington’s disease. Neurosci. Lett. 2007;411:98-103.

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220. 220.

     Hervás D., Fornés-Ferrer V., Gómez-Escribano A.P., Sequedo M.D., Peiró C., Millán J.M., Vázquez-Manrique R.P. Metformin intake associates with better cognitive function in patients with Huntington’s disease. PLoS ONE. 2017;12:1-11.

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221. 221.

     Wang J.Y.J. Regulation of cell death by the Abl tyrosine kinase. Oncogene. 2000;19:5643-5650.

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222. 222.

     Ko H.S., Lee Y., Shin J.H., Karuppagounder S.S., Gadad B.S., Koleske A.J., Pletnikova O., Troncoso J.C., Dawson V.L., Dawson T.M. Phosphorylation by the c-Abl protein tyrosine kinase inhibits parkin’s ubiquitination and protective function. Proc. Natl. Acad. Sci. USA. 2010;107:16691-16696.

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223. 223.

     Imam S.Z., Zhou Q., Yamamoto A., Valente A.J., Ali S.F., Bains M., Roberts J.L., Kahle P.J., Clark R.A., Li S. Novel regulation of Parkin function through c-Abl-mediated tyrosine phosphorylation: Implications for Parkinson’s disease. J. Neurosci. 2011;31:157-163.

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224. 224.

     Kumar M.J.V., Shah D., Giridharan M., Yadav N., Manjithaya R., Clement J.P. Spatiotemporal analysis of soluble aggregates and autophagy markers in the R6/2 mouse model. Sci. Rep. 2021;11:1-20.

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225. 225.

     Meng L., Zhao P., Hu Z., Ma W., Niu Y., Su J., Zhang Y., Nilotinib A. Tyrosine Kinase Inhibitor, Suppresses the Cell Growth and Triggers Autophagy in Papillary Thyroid Cancer. Anticancer Agents Med. Chem. 2021;21.

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226. 226.

     Yu H.C., Lin C.S., Tai W.T., Liu C.Y., Shiau C.W., Chen K.F. Nilotinib induces autophagy in hepatocellular carcinoma through AMPK activation. J. Biol. Chem. 2013;288:18249-18259.

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227. 227.

     Lonskaya I., Hebron M.L., Desforges N.M., Schachter J.B., Moussa C.E.H. Nilotinib-induced autophagic changes increase endogenous parkin level and ubiquitination, leading to amyloid clearance. J. Mol. Med. 2014;92:373-386.

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228. 228.

     Prerna K., Dubey V.K. Repurposing of FDA-approved drugs as autophagy inhibitors in tumor cells. J. Biomol. Struct. Dyn. 2021;1-12.

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229. 229.

     Pagan F., Hebron M., Valadez E.H., Torres-Yaghi Y., Huang X., Mills R.R., Wilmarth B.M., Howard H., Dunn C., Carlson A., et al. Nilotinib effects in Parkinson’s disease and dementia with lewy bodies. J. Parkinson’s Dis. 2016;6:503-517.

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230. 230.

     Hebron M.L., Lonskaya I., Moussa C.E.H. Nilotinib reverses loss of dopamine neurons and improvesmotorbehavior via autophagic degradation of α-synuclein in parkinson’s disease models. Hum. Mol. Genet. 2013;22:3315-3328.

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231. 231.

     Corrochano S., Renna M., Carter S., Chrobot N., Kent R., Stewart M., Cooper J., Brown S.D.M., Rubinsztein D.C., Acevedo-Arozena A. α-Synuclein levels modulate Huntington’s disease in mice. Hum. Mol. Genet. 2012;21:485-494.

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232. 232.

     Lansita J.A., Mease K.M., Qiu H., Yednock T., Sankaranarayanan S., Kramer S. Nonclinical Development of ANX005: A Humanized Anti-C1q Antibody for Treatment of Autoimmune and Neurodegenerative Diseases. Int. J. Toxicol. 2017;36:449-462.

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233. 233.
     Therapeutic Inhibition of the Complement System in Diseases of the Central Nervous System · 2019

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234. 234.

     Mestre T.A. Recent advances in the therapeutic development for Huntington disease. Park. Relat. Disord. 2019;59:125-130.

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235. 235.

     Tabrizi S.J., Leavitt B.R., Landwehrmeyer G.B., Wild E.J., Saft C., Barker R.A., Blair N.F., Craufurd D., Priller J., Rickards H., et al. Targeting Huntingtin Expression in Patients with Huntington’s Disease. N. Engl. J. Med. 2019;380:2307-2316.

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236. 236.

     Evers M.M., Miniarikova J., Juhas S., Vallès A., Bohuslavova B., Juhasova J., Skalnikova H.K., Vodicka P., Valekova I., Brouwers C. AAV5-miHTT Gene Therapy Demonstrates Broad Distribution and Strong Human Mutant Huntingtin Lowering in a Huntington’s Disease Minipig Model. Mol. Ther. 2018;26:2163-2177.

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237. 237.

     Spronck E.A., Brouwers C.C., Vallès A., Haan M., Petry H., Van Deventer S.J., Konstantinova P., Evers M.M. AAV5-miHTT Gene Therapy Demonstrates Sustained Huntingtin Lowering and Functional Improvement in Huntington Disease Mouse Models. Mol. Ther. Methods Clin. Dev. 2019;13:334-343.

     Not in our library10.1016/j.omtm.2019.03.002

238. 238.

     Miniarikova J., Zimmer V., Martier R., Brouwers C.C., Pythoud C., Richetin K., Rey M., Lubelski J., Evers M.M., Van Deventer S.J. AAV5-miHTT gene therapy demonstrates suppression of mutant huntingtin aggregation and neuronal dysfunction in a rat model of Huntington’s disease. Gene Ther. 2017;24:630-639.

     Not in our library10.1038/gt.2017.71

239. 239.

     Caron N.S., Southwell A.L., Brouwers C.C., Cengio L.D., Xie Y., Black H.F., Anderson L.M., Ko S., Zhu X., van Deventer S.J. Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease. Nucleic Acids Res. 2019;48:36-54.

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240. 240.

     Keskin S., Brouwers C.C., Sogorb-Gonzalez M., Martier R., Depla J.A., Vallès A., van Deventer S.J., Konstantinova P., Evers M.M. AAV5-miHTT lowers huntingtin mRNA and protein without Off-Target effects in patient-derived neuronal cultures and astrocytes. Mol. Ther. Methods Clin. Dev. 2019;15:275-284.

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241. 241.

     Spronck E.A., Vallès A., Lampen M.H., Montenegro-Miranda P.S., Keskin S., Heijink L., Evers M.M., Petry H., Deventer S., Konstantinova P., et al. Intrastriatal Administration of AAV5-miHTT in non-human primates and rats is well tolerated and results in miHTT transgene expression in key areas of Huntington disease pathology. Brain Sci. 2021;11:129.

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242. 242.

     Wadman M. Promising drug for Huntington disease fails in major trial. Science. 2021.

     Not in our library10.1126/science.abi6722

243. 243.

     Claustrat B., Leston J. Melatonin: Physiological effects in humans. Neurochirurgie. 2015;61:77-84.

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244. 244.

     Zhang P.Y., Liu X.J., Zhang Y.Q. Biotin-thiamine-responsive basal ganglia disease. Chinese J. Pediatr. 2018;56:462-464.

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245. 245.

     Ranen N.G., Peyser C.E., Coyle J.T., Bylsma F.W., Sherr M., Day L., Folstein M.F., Brandt J., Ross C.A., Folstein S.E. A controlled trial of idebenone in Huntington’s disease. Mov. Disord. 1996;11:549-554.

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246. 246.

     Wenceslau C.V., Kerkis I., Pompeia C., Haddad M.S. Pluripotent Stem Cells to Model and Treat Huntington’s Disease. Huntington’s Disease—Molecular Pathogenesis and Current Models. 2017.

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247. 247.

     Mu S., Wang J., Zhou G., Peng W., He Z., Zhao Z., Mo C.P., Qu J., Zhang J. Transplantation of induced pluripotent stem cells improves functional recovery in Huntington’s disease rat model. PLoS ONE. 2014;9:101185.

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248. 248.

     Benabid A.L., Pollak P., Louveau A., Henry S., De Rougemont J. Combined (thalamotomy and stimulation) stereotactic surgery of the vim thalamic nucleus for bilateral parkinson disease. Stereotact. Funct. Neurosurg. 1987;50:344-346.

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249. 249.

     Watson Alberts W., Wright E.W., Levin G., Feinstein B., Mueller M. Threshold stimulation of the lateral thalamus and globus pallidus in the waking human. Electroencephalogr. Clin. Neurophysiol. 1961;13:68-74.

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250. 250.

     Sem-Jacobsen C.W. Depth-electrographic observations related to Parkinson’s disease. Recording and electrical stimulation in the area around the third ventricle. J. Neurosurg. 1966;24:388-402.

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251. 251.

     Mundinger F. New stereotactic treatment of spasmodic torticollis with a brain stimulation system. Med. Klin. 1977;72:1982-1986.

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252. 252.

     Herrington T.M., Cheng J.J., Eskandar E.N. Mechanisms of deep brain stimulation. J. Neurophysiol. 2016;115:19-38.

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253. 253.

     Fasano A., Mazzone P., Piano C., Quaranta D., Soleti F., Bentivoglio A.R. GPi-DBS in Huntington’s disease: Results on motor function and cognition in a 72-year-old case. Mov. Disord. 2008;23:1289-1292.

     Not in our library10.1002/mds.22116

254. 254.

     Moro E., Lang A.E., Strafella A.P., Poon Y.Y.W., Arango P.M., Dagher A., Hutchison W.D., Lozano A.M. Bilateral globus pallidus stimulation for Huntington’s disease. Ann. Neurol. 2004;56:290-294.

     Not in our library10.1002/ana.20183

255. 255.

     Gonzalez V., Cif L., Biolsi B., Garcia-Ptacek S., Seychelles A., Sanrey E., Descours I., Coubes C., Ribeiro De Moura A.M., Corlobe A., et al. Deep brain stimulation for Huntington’s disease: Long-term results of a prospective open-label study: Clinical article. J. Neurosurg. 2014;121:114-122.

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256. 256.

     Gruber D., Kuhn A.A., Schoenecker T., Kopp U.A., Kivi A., Huebl J., Lobsien E., Mueller B., Schneider G.H., Kupsch A. Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: Case report and review of the literature. J. Neural Transm. 2014;121:1303-1312.

     Not in our library10.1007/s00702-014-1201-7

257. 257.

     Fawcett A.P., Moro E., Lang A.E., Lozano A.M., Hutchison W.D. Pallidal deep brain stimulation influences both reflexive and voluntary saccades in Huntington’s disease. Mov. Disord. 2005;20:371-377.

     Not in our library10.1002/mds.20356

258. 258.

     Hebb M.O., Garcia R., Gaudet P., Mendez I.M. Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington’s disease: Technical case report. Neurosurgery. 2006;58.

     Not in our library10.1227/01.neu.0000195068.19801.18

259. 259.

     Garcia-Ruiz P.J., Ayerbe J., del Val J., Herranz A. Deep brain stimulation in disabling involuntary vocalization associated with Huntington’s disease. Parkinsonism Relat. Disord. 2012;18:803-804.

     Not in our library10.1016/j.parkreldis.2012.03.005

260. 260.

     Biolsi B., Cif L., El Fertit H., Robles S.G., Coubes P. Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus: Case report. J. Neurosurg. 2008;109:130-132.

     Not in our library10.3171/jns/2008/109/7/0130

261. 261.

     Kang G.A., Heath S., Rothlind J., Starr P.A. Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington’s disease. J. Neurol. Neurosurg. Psychiatry. 2011;82:272-277.

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262. 262.

     Spielberger S., Hotter A., Wolf E., Eisner W., Müller J., Poewe W., Seppi K. Deep brain stimulation in Huntington’s disease: A 4-year follow-up case report. Mov. Disord. 2012;27:806-807.

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263. 263.

     López-Sendón Moreno J.L., García-Caldentey J., Regidor I., del Álamo M., García de Yébenes J. A 5-year follow-up of deep brain stimulation in Huntington’s disease. Parkinsonism Relat. Disord. 2014;20:260-261.

     Not in our library10.1016/j.parkreldis.2013.11.007

264. 264.

     Cislaghi G., Capiluppi E., Saleh C., Romano L., Servello D., Mariani C., Porta M. Bilateral Globus Pallidus Stimulation in Westphal Variant of Huntington Disease. Neuromodulation Technol. Neural Interface. 2014;17:502-505.

     Not in our library10.1111/ner.12098

265. 265.

     Khalil H., Quinn L., Van Deursen R., Dawes H., Playle R., Rosser A., Busse M. What effect does a structured home-based exercise programme have on people with Huntington’s disease? A randomized, controlled pilot study. Clin. Rehabil. 2013;27:646-658.

     Not in our library10.1177/0269215512473762

266. 266.

     Quinn L., Debono K., Dawes H., Rosser A.E., Nemeth A.H., Rickards H., Tabrizi S.J., Quarrell O., Trender-Gerhard I., Kelson M.J., et al. Task-specific training in Huntington disease: A randomized controlled feasibility trial. Phys. Ther. 2014;94:1555-1568.

     Not in our library10.2522/ptj.20140123

267. 267.

     Quinn L., Hamana K., Kelson M., Dawes H., Collett J., Townson J., Roos R., van der Plas A.A., Reilmann R., Frich J.C., et al. A randomized, controlled trial of a multi-modal exercise intervention in Huntington’s disease. Park. Relat. Disord. 2016;31:46-52.

     Not in our library10.1016/j.parkreldis.2016.06.023

268. 268.

     Busse M., Quinn L., Debono K., Jones K., Collett J., Playle R., Kelly M., Simpson S., Backx K., Wasley D., et al. A randomized feasibility study of a 12-week community-based exercise program for people with Huntington’s disease. J. Neurol. Phys. Ther. 2013;37:149-158.

     Not in our library10.1097/npt.0000000000000016

269. 269.

     Kloos A.D., Fritz N.E., Kostyk S.K., Young G.S., Kegelmeyer D.A. Video game play (Dance Dance Revolution) as a potential exercise therapy in Huntington’s disease: A controlled clinical trial. Clin. Rehabil. 2013;27:972-982.

     Not in our library10.1177/0269215513487235

270. 270.

     Fritz N., Rao A.K., Kegelmeyer D., Kloos A., Busse M., Hartel L., Carrier J., Quinn L. Physical Therapy and Exercise Interventions in Huntington’s Disease: A Mixed Methods Systematic Review. J. Huntingtons. Dis. 2017;6:217-236.

     Not in our library10.3233/jhd-170260

271. 271.

     Drew C.J.G., Quinn L., Hamana K., Williams-Thomas R., Marsh L., Dimitropoulou P., Playle R., Griffin B.A., Kelson M., Schubert R., et al. Physical Activity and Exercise Outcomes in Huntington Disease (PACE-HD): Protocol for a 12-Month Trial Within Cohort Evaluation of a Physical Activity Intervention in People with Huntington Disease. Phys. Ther. 2019;99:1201-1210.

     Not in our library10.1093/ptj/pzz075

272. 272.

     Zuccato C., Cattaneo E. Brain-derived neurotrophic factor in neurodegenerative diseases. Nat. Rev. Neurol. 2009;5:311-322.

     Not in our library10.1038/nrneurol.2009.54

273. 273.

     Elifani F., Amico E., Pepe G., Capocci L., Castaldo S., Rosa P., Montano E., Pollice A., Madonna M., Filosa S., et al. Curcumin dietary supplementation ameliorates disease phenotype in an animal model of Huntington’s disease. Hum. Mol. Genet. 2019;28:4012-4021.

     Not in our library10.1093/hmg/ddz247

274. 274.

     Spires T.L., Grote H.E., Varshney N.K., Cordery P.M., Van Dellen A., Blakemore C., Hannan A.J. Environmental Enrichment Rescues Protein Deficits in a Mouse Model of Huntington’s Disease, Indicating a Possible Disease Mechanism. J. Neurosci. 2004;24:2270-2276.

     Not in our library10.1523/jneurosci.1658-03.2004

275. 275.

     Couly S., Carles A., Denus M., Benigno-Anton L., Maschat F., Maurice T. Exposure of R6/2 mice in an enriched environment augments P42 therapy efficacy on Huntington’s disease progression. Neuropharmacology. 2021;186:108467.

     Not in our library10.1016/j.neuropharm.2021.108467

276. 276.

     Kim Y.-M., Ji E.-S., Kim S.-H., Kim T.-W., Ko I.-G., Jin J.-J., Kim C.-J., Kim T.-W., Kim D.-H. Treadmill exercise improves short-term memory by enhancing hippocampal cell proliferation in quinolinic acid-induced Huntington’s disease rats. J. Exerc. Rehabil. 2015;11:5-11.

     Not in our library10.12965/jer.150182

277. 277.

     Zajac M.S., Pang T.Y.C., Wong N., Weinrich B., Leang L.S.K., Craig J.M., Saffery R., Hannan A.J. Wheel running and environmental enrichment differentially modify exon-specific BDNF expression in the hippocampus of wild-type and pre-motor symptomatic male and female Huntington’s disease mice. Hippocampus. 2010;20:621-636.

     Not in our library10.1002/hipo.20658

278. 278.

     Canals J.M., Pineda J.R., Torres-Peraza J.F., Bosch M., Martín-Ibañez R., Muñoz M.T., Mengod G., Ernfors P., Alberch J. Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington’s disease. J. Neurosci. 2004;24:7727-7739.

     Not in our library10.1523/jneurosci.1197-04.2004

279. 279.

     Cho S.R., Benraiss A., Chmielnicki E., Samdani A., Economides A., Goldman S.A. Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease. J. Clin. Investig. 2007;117:2889-2902.

     Not in our library10.1172/jci31778

280. 280.

     Kells A.P., Fong D.M., Dragunow M., During M.J., Young D., Connor B. AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease. Mol. Ther. 2004;9:682-688.

     Not in our library10.1016/j.ymthe.2004.02.016

281. 281.

     Frim D.M., Uhler T.A., Short M.P., Ezzedine Z.D., Klagsbrun M., Breakefield X.O., Isacson O. Effects of biologically delivered NGF, bdnf and BFGF on striatal excitotoxic lesions. Neuroreport. 1993;4:367-370.

     Not in our library10.1097/00001756-199304000-00006

282. 282.

     Martínez-Serrano A., Björklund A. Protection of the neostriatum against excitotoxic damage by neurotrophin-producing, genetically modified neural stem cells. J. Neurosci. 1996;16:4604-4616.

     Not in our library10.1523/jneurosci.16-15-04604.1996

283. 283.

     Pérez-Navarro E., Alberch J., Neveu I., Arenas E. Brain-derived neurotrophic factor, neurotrophin-3 and neurotrophin-4/5 differentially regulate the phenotype and prevent degenerative changes in striatal projection neurons after excitotoxicity in vivo. Neuroscience. 1999;91:1257-1264.

     Not in our library10.1016/s0306-4522(98)00723-4

284. 284.

     Pérez-Navarro E., Canudas A.A., Akerud P., Alberch J., Arenas E. Brain-derived neurotrophic factor, neurotrophin-3, and neurotrophin-4/5 prevent the death of striatal projection neurons in a rodent model of Huntington’s disease. J. Neurochem. 2000;75:2190-2199.

     Not in our library10.1046/j.1471-4159.2000.0752190.x

285. 285.

     Ryu J.K., Kim J., Cho S.J., Hatori K., Nagai A., Choi H.B., Lee M.C., McLarnon J.G., Kim S.U. Proactive transplantation of human neural stem cells prevents degeneration of striatal neurons in a rat model of Huntington disease. Neurobiol. Dis. 2004;16:68-77.

     Not in our library10.1016/j.nbd.2004.01.016

286. 286.

     Sari Y. Potential Drugs and Methods for Preventing or Delaying the Progression of Huntington’s Disease. Recent Pat. CNS Drug Discov. 2011;6:80-90.

     Not in our library10.2174/157488911795933884

287. 287.

     Gill S.S., Patel N.K., Hotton G.R., O’Sullivan K., McCarter R., Bunnage M., Brooks D.J., Svendsen C.N., Heywood P. Direct brain infusion of glial cell line-derived neurotrophic factor in Parkinson disease. Nat. Med. 2003;9:589-595.

     Not in our library10.1038/nm850

288. 288.

     Arregui L., Benítez J.A., Razgado L.F., Vergara P., Segovia J. Adenoviral astrocyte-specific expression of BDNF in the striata of mice transgenic for Huntington’s disease delays the onset of the motor phenotype. Cell. Mol. Neurobiol. 2011;31:1229-1243.

     Not in our library10.1007/s10571-011-9725-y

289. 289.

     Teng H.K., Teng K.K., Lee R., Wright S., Tevar S., Almeida R.D., Kermani P., Torkin R., Chen Z.Y., Lee F.S., et al. ProBDNF induces neuronal apoptosis via activation of a receptor complex of p75NTR and sortilin. J. Neurosci. 2005;25:5455-5463.

     Not in our library10.1523/jneurosci.5123-04.2005

290. 290.

     Hacein-Bey-Abina S., Garrigue A., Wang G.P., Soulier J., Lim A., Morillon E., Clappier E., Caccavelli L., Delabesse E., Beldjord K., et al. Insertional oncogenesis in 4 patients after retrovirus-mediated gene therapy of SCID-X1. J. Clin. Investig. 2008;118:3132-3142.

     Not in our library10.1172/jci35700

291. 291.

     Biffi A., Naldini L. Gene therapy of storage disorders by retroviral and lentiviral vectors. Hum. Gene Ther. 2005;16:1133-1142.

     Not in our library10.1089/hum.2005.16.1133

292. 292.

     Yáñez-Muñoz R.J., Balaggan K.S., MacNeil A., Howe S.J., Schmidt M., Smith A.J., Buch P., MacLaren R.E., Anderson P.N., Barker S.E., et al. Effective gene therapy with nonintegrating lentiviral vectors. Nat. Med. 2006;12:348-353.

     Not in our library10.1038/nm1365

293. 293.

     Conti L., Cattaneo E. Neural stem cell systems: Physiological players or in vitro entities? Nat. Rev. Neurosci. 2010;11:176-187.

     Not in our library10.1038/nrn2761

294. 294.

     Rossi F., Cattaneo E. Neural stem cell therapy for neurological diseases: Dreams and reality. Nat. Rev. Neurosci. 2002;3:401-409.

     Not in our library10.1038/nrn809

295. 295.

     Schiefer J., Landwehrmeyer G.B., Lüesse H.G., Sprünken A., Puls C., Milkereit A., Milkereit E., Kosinski C.M. Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington’s disease. Mov. Disord. 2002;17:748-757.

     Not in our library10.1002/mds.10229

296. 296.

     Hockly E., Tse J., Barker A.L., Moolman D.L., Beunard J.L., Revington A.P., Holt K., Sunshine S., Moffitt H., Sathasivam K., et al. Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington’s disease. Neurobiol. Dis. 2006;21:228-236.

     Not in our library10.1016/j.nbd.2005.07.007

297. 297.

     Lesort M., Lee M., Tucholski J., Johnson G.V.W. Cystamine inhibits caspase activity: Implications for the treatment of polyglutamine disorders. J. Biol. Chem. 2003;278:3825-3830.

     Not in our library10.1074/jbc.m205812200

298. 298.

     Fox J.H., Barber D.S., Singh B., Zucker B., Swindell M.K., Norflus F., Buzescu R., Chopra R., Ferrante R.J., Kazantsev A., et al. Cystamine increases L-cysteine levels in Huntington’s disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J. Neurochem. 2004;91:413-422.

     Not in our library10.1111/j.1471-4159.2004.02726.x

299. 299.

     Borrell-Pagès M., Canals J.M., Cordelières F.P., Parker J.A., Pineda J.R., Grange G., Bryson E.A., Guillermier M., Hirsch E., Hantraye P., et al. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J. Clin. Investig. 2006;116:1410-1424.

     Not in our library10.1172/jci27607

300. 300.

     Powrozek T., Sari Y., Singh R., Zhou F. Neurotransmitters and Substances of Abuse: Effects on Adult Neurogenesis. Curr. Neurovasc. Res. 2005;1:251-260.

     Not in our library10.2174/1567202043362225

301. 301.

     Gewirtz J.C., Chen A.C., Terwilliger R., Duman R.C., Marek G.J. Modulation of DOI-induced increases in cortical BDNF expression by group II mGlu receptors. Pharmacol. Biochem. Behav. 2002;73:317-326.

     Not in our library10.1016/s0091-3057(02)00844-4

302. 302.

     Vaidya V.A., Marek G.J., Aghajanian G.K., Duman R.S. 5-HT(2A) receptor-mediated regulation of brain-derived neurotrophic factor mRNA in the hippocampus and the neocortex. J. Neurosci. 1997;17:2785-2795.

     Not in our library10.1523/jneurosci.17-08-02785.1997

303. 303.

     Vaidya V.A., Terwilliger R.M.Z., Duman R.S. Role of 5-HT(2a) receptors in the stress-induced down-regulation of brain-derived neurotrophic factor expression in rat hippocampus. Neurosci. Lett. 1999;262:1-4.

     Not in our library10.1016/s0304-3940(99)00006-3

304. 304.

     Duman R.S. Novel therapeutic approaches beyond the serotonin receptor. Biol. Psychiatry. 1998;44:324-335.

     Not in our library10.1016/s0006-3223(98)00031-6

305. 305.

     Duan W., Guo Z., Jiang H., Ladenheim B., Xu X., Cadet J.L., Mattson M.P. Paroxetine Retards Disease Onset and Progression in Huntingtin Mutant Mice. Ann. Neurol. 2004;55:590-594.

     Not in our library10.1002/ana.20075

306. 306.

     Grote H.E., Bull N.D., Howard M.L., Van Dellen A., Blakemore C., Bartlett P.F., Hannan A.J. Cognitive disorders and neurogenesis deficits in Huntington’s disease mice are rescued by fluoxetine. Eur. J. Neurosci. 2005;22:2081-2088.

     Not in our library10.1111/j.1460-9568.2005.04365.x

307. 307.

     Duan W., Peng Q., Masuda N., Ford E., Tryggestad E., Ladenheim B., Zhao M., Cadet J.L., Wong J., Ross C.A. Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington’s disease. Neurobiol. Dis. 2008;30:312-322.

     Not in our library10.1016/j.nbd.2008.01.015

308. 308.

     Slaughter J.R., Martens M.P., Slaughter K.A. Depression and Huntington’s disease: Prevalence, clinical manifestations, etiology, and treatment. CNS Spectr. 2001;6:306-326.

     Not in our library10.1017/s109285290002201x

309. 309.

     Cong W.N., Chadwick W., Wang R., Daimon C.M., Cai H., Amma J., Wood W.H., Becker K.G., Martin B., Maudsley S. Amitriptyline improves motor function via enhanced neurotrophin signaling and mitochondrial functions in the murine N171-82Q Huntington disease model. J. Biol. Chem. 2015;290:2728-2743.

     Not in our library10.1074/jbc.m114.588608

310. 310.

     Ellrichmann G., Blusch A., Fatoba O., Brunner J., Hayardeny L., Hayden M., Sehr D., Winklhofer K.F., Saft C., Gold R. Laquinimod treatment in the R6/2 mouse model. Sci. Rep. 2017;7.

     Not in our library10.1038/s41598-017-04990-1

311. 311.

     Garcia-Miralles M., Yusof N.A.B.M., Tan J.Y., Radulescu C.I., Sidik H., Tan L.J., Belinson H., Zach N., Hayden M.R., Pouladi M.A. Laquinimod Treatment Improves Myelination Deficits at the Transcriptional and Ultrastructural Levels in the YAC128 Mouse Model of Huntington Disease. Mol. Neurobiol. 2019;56:4464-4478.

     Not in our library10.1007/s12035-018-1393-1

312. 312.

     Corey-Bloom J., Aikin A.M., Gutierrez A.M., Nadhem J.S., Howell T.L., Thomas E.A. Beneficial effects of glatiramer acetate in Huntington’s disease mouse models: Evidence for BDNF-elevating and immunomodulatory mechanisms. Brain Res. 2017;1673:102-110.

     Not in our library10.1016/j.brainres.2017.08.013

313. 313.

     Reick C., Ellrichmann G., Tsai T., Lee D.H., Wiese S., Gold R., Saft C., Linker R.A. Expression of brain-derived neurotrophic factor in astrocytes—Beneficial effects of glatiramer acetate in the R6/2 and YAC128 mouse models of Huntington’s disease. Exp. Neurol. 2016;285:12-23.

     Not in our library10.1016/j.expneurol.2016.08.012

314. 314.
     Prostaglandin E2 EP2 activation reduces memory decline in R6/1 mouse model of Huntington's disease by the induction of BDNF-dependent synaptic plasticity · 2016

     Not checked
315. 315.

     Da Fonsêca V.S., da Silva Colla A.R., de Paula Nascimento-Castro C., Plácido E., Rosa J.M., Farina M., Gil-Mohapel J., Rodrigues A.L.S., Brocardo P.S. Brain-Derived Neurotrophic Factor Prevents Depressive-Like Behaviors in Early-Symptomatic YAC128 Huntington’s Disease Mice. Mol. Neurobiol. 2018;55:7201-7215.

     Not in our library10.1007/s12035-018-0890-6

316. 316.

     Cheng S., Tereshchenko J., Zimmer V., Vachey G., Pythoud C., Rey M., Liefhebber J., Raina A., Streit F., Mazur A., et al. Therapeutic efficacy of regulable GDNF expression for Huntington’s and Parkinson’s disease by a high-induction, background-free “GeneSwitch” vector. Exp. Neurol. 2018;309:79-90.

     Not in our library10.1016/j.expneurol.2018.07.017

317. 317.

     Cabezas-Llobet N., Vidal-Sancho L., Masana M., Fournier A., Alberch J., Vaudry D., Xifró X. Pituitary Adenylate Cyclase-Activating Polypeptide (PACAP) Enhances Hippocampal Synaptic Plasticity and Improves Memory Performance in Huntington’s Disease. Mol. Neurobiol. 2018;55:8263-8277.

     Not in our library10.1007/s12035-018-0972-5

318. 318.

     Fatoba O., Kloster E., Reick C., Saft C., Gold R., Epplen J.T., Arning L., Ellrichmann G. Activation of NPY-Y2 receptors ameliorates disease pathology in the R6/2 mouse and PC12 cell models of Huntington’s disease. Exp. Neurol. 2018;302:112-128.

     Not in our library10.1016/j.expneurol.2018.01.001

319. 319.

     Allen S.J., Watson J.J., Shoemark D.K., Barua N.U., Patel N.K. GDNF, NGF and BDNF as therapeutic options for neurodegeneration. Pharmacol. Ther. 2013;138:155-175.

     Not in our library10.1016/j.pharmthera.2013.01.004

320. 320.

     Mandel R.J., Spratt S.K., Snyder R.O., Leff S.E. Midbrain injection of recombinant adeno-associated virus encoding rat glial cell line-derived neurotrophic factor protects nigral neurons in a progressive 6-hydroxydopamine-induced degeneration model of Parkinson’s disease in rats. Proc. Natl. Acad. Sci. USA. 1997;94:14083-14088.

     Not in our library10.1073/pnas.94.25.14083

321. 321.

     Rahimi-Movaghar V., Yan H.Q., Li Y., Ma X., Akbarian F., Dixon C.E. Increased expression of glial cell line-derived neurotrophic factor in rat brain after traumatic brain injury. Acta Med. Iran. 2005;43:7-10.

     Not in our library

322. 322.

     Araujo D.M., Hilt D.C. Glial cell line-derived neurotrophic factor attenuates the excitotoxin- induced behavioral and neurochemical deficits in a rodent model of Huntington’s disease. Neuroscience. 1997;81:1099-1110.

     Not in our library10.1016/s0306-4522(97)00079-1

323. 323.

     McBride J.L., During M.J., Wuu J., Chen E.Y., Leurgans S.E., Kordower J.H. Structural and functional neuroprotection in a rat model of Huntington’s disease by viral gene transfer of GDNF. Exp. Neurol. 2003;181:213-223.

     Not in our library10.1016/s0014-4886(03)00044-x

324. 324.

     McBride J.L., Ramaswamy S., Gasmi M., Bartus R.T., Herzog C.D., Brandon E.P., Zhou L., Pitzer M.R., Berry-Kravis E.M., Kordower J.H. Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington’s disease. Proc. Natl. Acad. Sci. USA. 2006;103:9345-9350.

     Not in our library10.1073/pnas.0508875103

325. 325.

     Popovic N., Maingay M., Kirik D., Brundin P. Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes. Exp. Neurol. 2005;193:65-74.

     Not in our library10.1016/j.expneurol.2004.12.009

326. 326.

     Skaper S.D., Selak I., Manthorpe M., Varon S. Chemically defined requirements for the survival of cultured 8-day chick embryo ciliary ganglion neurons. Brain Res. 1984;302:281-290.

     Not in our library10.1016/0006-8993(84)90241-5

327. 327.

     Pasquin S., Sharma M., Gauchat J.F. Ciliary neurotrophic factor (CNTF): New facets of an old molecule for treating neurodegenerative and metabolic syndrome pathologies. Cytokine Growth Factor Rev. 2015;26:507-515.

     Not in our library10.1016/j.cytogfr.2015.07.007

328. 328.

     Anderson K.D., Panayotatos N., Corcoran T.L., Lindsay R.M., Wiegand S.J. Ciliary neurotrophic factor protects striatal output neurons in an animal model of Huntington disease. Proc. Natl. Acad. Sci. USA. 1996;93:7346-7351.

     Not in our library10.1073/pnas.93.14.7346

329. 329.

     Emerich D.F., Winn S.R., Hantraye P.M., Peschanski M., Chen E.Y., Chu Y., McDermott P., Baetge E.E., Kordower J.H. Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington’s disease. Nature. 1997;386:395-399.

     Not in our library10.1038/386395a0

330. 330.

     De Almeida L.P., Zala D., Aebischer P., Déglon N. Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington’s disease. Neurobiol. Dis. 2001;8:433-446.

     Not in our library10.1006/nbdi.2001.0388

331. 331.

     Ramirez B.U., Retamal L., Vergara C. Ciliary neurotrophic factor (CNTF) affects the excitable and contractile properties of innervated skeletal muscles. Biol. Res. 2003;36:303-312.

     Not in our library10.4067/s0716-97602003000300003

332. 332.

     Bongioanni P., Reali C., Sogos V. Ciliary neurotrophic factor (CNTF) for amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst. Rev. 2004;3.

     Not in our library10.1002/14651858.cd004302.pub2

333. 333.

     Boucher J., Tseng Y.H., Kahn C.R. Insulin and insulin-like growth factor-1 receptors act as ligand-specific amplitude modulators of a common pathway regulating gene transcription. J. Biol. Chem. 2010;285:17235-17245.

     Not in our library10.1074/jbc.m110.118620

334. 334.

     Duarte A.I., Petit G.H., Ranganathan S., Li J.Y., Oliveira C.R., Brundin P., Björkqvist M., Rego A.C. IGF-1 protects against diabetic features in an in vivo model of Huntington’s disease. Exp. Neurol. 2011;231:314-319.

     Not in our library10.1016/j.expneurol.2011.06.016

335. 335.

     Lopes C., Ribeiro M., Duarte A.I., Humbert S., Saudou F., Pereira De Almeida L., Hayden M., Rego A.C. IGF-1 intranasal administration rescues Huntington’s disease phenotypes in YAC128 mice. Mol. Neurobiol. 2014;49:1126-1142.

     Not in our library10.1007/s12035-013-8585-5

336. 336.

     McCloskey D.P., Hintz T.M., Scharfman H.E. Modulation of vascular endothelial growth factor (VEGF) expression in motor neurons and its electrophysiological effects. Brain Res. Bull. 2008;76:36-44.

     Not in our library10.1016/j.brainresbull.2007.11.018

337. 337.

     Ellison S.M., Trabalza A., Tisato V., Pazarentzos E., Lee S., Papadaki V., Goniotaki D., Morgan S., Mirzaei N., Mazarakis N.D. Dose-dependent neuroprotection of VEGF165 in Huntington’s disease striatum. Mol. Ther. 2013;21:1862-1875.

     Not in our library10.1038/mt.2013.132

338. 338.

     Ji C.H., Kwon Y.T. Crosstalk and Interplay between the Ubiquitin-Proteasome System and Autophagy. Mol. Cells. 2017;40:441-449.

     Not in our library10.14348/molcells.2017.0115

339. 339.

     Pohl C., Dikic I. Cellular quality control by the ubiquitin-proteasome system and autophagy. Science. 2019;366:818-822.

     Not in our library10.1126/science.aax3769

340. 340.

     Wang Y., Le W.-D. Autophagy and Ubiquitin-Proteasome System. Advances in Experimental Medicine and Biology. 2019;Volume 1206:527-550.

     Not in our library10.1007/978-981-15-0602-4_25

341. 341.

     Şentürk M., Lin G., Zuo Z., Mao D., Watson E., Mikos A.G., Bellen H.J. Ubiquilins regulate autophagic flux through mTOR signalling and lysosomal acidification. Nat. Cell Biol. 2019;21:384-396.

     Not in our library10.1038/s41556-019-0281-x

342. 342.

     Safren N., El Ayadi A., Chang L., Terrillion C.E., Gould T.D., Boehning D.F., Monteiro M.J. Ubiquilin-1 overexpression increases the lifespan and delays accumulation of huntingtin aggregates in the R6/2 mouse model of huntington’s disease. PLoS ONE. 2014;9:e87513.

     Not in our library10.1371/journal.pone.0087513

343. 343.

     Weber J.J., Pereira Sena P., Singer E., Nguyen H.P. Killing Two Angry Birds with One Stone: Autophagy Activation by Inhibiting Calpains in Neurodegenerative Diseases and Beyond. Biomed. Res. Int. 2019;2019:1-13.

     Not in our library10.1155/2019/4741252

344. 344.

     Menzies F.M., Garcia-Arencibia M., Imarisio S., O’Sullivan N.C., Ricketts T., Kent B.A., Rao M.V., Lam W., Green-Thompson Z.W., Nixon R.A., et al. Calpain inhibition mediates autophagy-dependent protection against polyglutamine toxicity. Cell Death Differ. 2015;22:433-444.

     Not in our library10.1038/cdd.2014.151

345. 345.

     Cortes C.J., La Spada A.R. TFEB dysregulation as a driver of autophagy dysfunction in neurodegenerative disease: Molecular mechanisms, cellular processes, and emerging therapeutic opportunities. Neurobiol. Dis. 2019;122:83-93.

     Not in our library10.1016/j.nbd.2018.05.012

346. 346.

     Vodicka P., Chase K., Iuliano M., Tousley A., Valentine D.T., Sapp E., Kegel-Gleason K.B., Sena-Esteves M., Aronin N., Difiglia M. Autophagy Activation by Transcription Factor EB (TFEB) in Striatum of HD Q175/Q7 Mice. J. Huntingtons. Dis. 2016;5:249-260.

     Not in our library10.3233/jhd-160211

347. 347.
     Mechanism of neuroprotection by trehalose: controversy surrounding autophagy induction · 2018

     No notice
348. 348.

     Tanaka M., Machida Y., Niu S., Ikeda T., Jana N.R., Doi H., Kurosawa M., Nekooki M., Nukina N. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med. 2004;10:148-154.

     Not in our library10.1038/nm985

349. 349.

     Rose C., Menzies F.M., Renna M., Acevedo-Arozena A., Corrochano S., Sadiq O., Brown S.D., Rubinsztein D.C. Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington’s disease. Hum. Mol. Genet. 2010;19:2144-2153.

     Not in our library10.1093/hmg/ddq093

350. 350.

     Underwood B.R., Green-Thompson Z.W., Pugh P.J., Lazic S.E., Mason S.L., Griffin J., Jones P.S., Rowe J.B., Rubinsztein D.C., Barker R.A. An open-label study to assess the feasibility and tolerability of rilmenidine for the treatment of Huntington’s disease. J. Neurol. 2017;264:2457-2463.

     Not in our library10.1007/s00415-017-8647-0

351. 351.

     Jiang W., Wei W., Gaertig M.A., Li S., Li X.J. Therapeutic effect of berberine on Huntington’s disease transgenic mouse model. PLoS ONE. 2015;10:e0134142.

     Not in our library10.1371/journal.pone.0134142

352. 352.

     Zhang T., Dong K., Liang W., Xu D., Xia H., Geng J., Najafov A., Liu M., Li Y., Han X., et al. G-protein Coupled Receptors Regulate Autophagy by ZBTB16-mediated Ubiquitination and Proteasomal Degradation of Adaptor Protein Atg14L. Elife. 2015;2015:e06734.

     Not in our library10.7554/elife.06734

353. 353.

     Jeon J., Kim W., Jang J., Isacson O., Seo H. Gene therapy by proteasome activator, PA28γ, improves motor coordination and proteasome function in Huntington’s disease YAC128 mice. Neuroscience. 2016;324:20-28.

     Not in our library10.1016/j.neuroscience.2016.02.054

354. 354.

     Valionyte E., Yang Y., Roberts S.L., Kelly J., Lu B., Luo S. Lowering Mutant Huntingtin Levels and Toxicity: Autophagy-Endolysosome Pathways in Huntington’s Disease. J. Mol. Biol. 2020;432:2673-2691.

     Not in our library10.1016/j.jmb.2019.11.012

355. 355.

     Neo S.H., Tang B.L. Sirtuins as Modifiers of Huntington’s Disease (HD) Pathology. Progress in Molecular Biology and Translational Science. 2018;Volume 154:105-145.

     Not in our library10.1016/bs.pmbts.2017.11.013

356. 356.

     Duan W. Targeting Sirtuin-1 in Huntington’s Disease: Rationale and Current Status. CNS Drugs. 2013;27:345-352.

     Not in our library10.1007/s40263-013-0055-0

357. 357.
     Mechanisms and disease implications of sirtuin-mediated autophagic regulation · 2019

     Not checked
358. 358.

     Jeong H., Cohen D.E., Cui L., Supinski A., Savas J.N., Mazzulli J.R., Yates J.R., Bordone L., Guarente L., Krainc D. Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway. Nat. Med. 2012;18:159-165.

     Not in our library10.1038/nm.2559

359. 359.

     Jiang M., Wang J., Fu J., Du L., Jeong H., West T., Xiang L., Peng Q., Hou Z., Cai H., et al. Neuroprotective role of Sirt1 in mammalian models of Huntington’s disease through activation of multiple Sirt1 targets. Nat. Med. 2012;18:153-158.

     Not in our library10.1038/nm.2558

360. 360.

     Jiang M., Zheng J., Peng Q., Hou Z., Zhang J., Mori S., Ellis J.L., Vlasuk G.P., Fries H., Suri V., et al. Sirtuin 1 activator SRT2104 protects Huntington’s disease mice. Ann. Clin. Transl. Neurol. 2014;1:1047-1052.

     Not in our library10.1002/acn3.135

361. 361.

     Lee M., Ban J.J., Chung J.Y., Im W., Kim M. Amelioration of huntington’s disease phenotypes by beta-lapachone is associated with increases in sirt1 expression, creb phosphorylation and pgc-1α deacetylation. PLoS ONE. 2018;13:e0195968.

     Not in our library10.1371/journal.pone.0195968

362. 362.

     Naia L., Rosenstock T.R., Oliveira A.M., Oliveira-Sousa S.I., Caldeira G.L., Carmo C., Laço M.N., Hayden M.R., Oliveira C.R., Rego A.C. Comparative Mitochondrial-Based Protective Effects of Resveratrol and Nicotinamide in Huntington’s Disease Models. Mol. Neurobiol. 2017;54:5385-5399.

     Not in our library10.1007/s12035-016-0048-3

363. 363.

     Smith M.R., Syed A., Lukacsovich T., Purcell J., Barbaro B.A., Worthge S.A., Wei S.R., Pollio G., Magnoni L., Scali C., et al. A potent and selective sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of huntington’s disease. Hum. Mol. Genet. 2014;23:2995-3007.

     Not in our library10.1093/hmg/ddu010

364. 364.

     Hathorn T., Snyder-Keller A., Messer A. Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington’s disease. Neurobiol. Dis. 2011;41:43-50.

     Not in our library10.1016/j.nbd.2010.08.017

365. 365.

     Tulino R., Benjamin A.C., Jolinon N., Smith D.L., Chini E.N., Carnemolla A., Bates G.P. SIRT1 activity is linked to its brain region-specific phosphorylation and is impaired in Huntington’s disease mice. PLoS ONE. 2016;11:e0145425.

     Not in our library10.1371/journal.pone.0145425

366. 366.

     Baldo B., Gabery S., Soylu-Kucharz R., Cheong R.Y., Henningsen J.B., Englund E., McLean C., Kirik D., Halliday G., Petersén H.A. SIRT1 is increased in affected brain regions and hypothalamic metabolic pathways are altered in Huntington disease. Neuropathol. Appl. Neurobiol. 2019;45:361-379.

     Not in our library10.1111/nan.12514

367. 367.

     Luthi-Carter R., Taylor D.M., Pallos J., Lambert E., Amore A., Parker A., Moffitt H., Smith D.L., Runne H., Gokce O., et al. SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc. Natl. Acad. Sci. USA. 2010;107:7927-7932.

     Not in our library10.1073/pnas.1002924107

368. 368.

     Chopra V., Quinti L., Kim J., Vollor L., Narayanan K.L., Edgerly C., Cipicchio P.M., Lauver M.A., Choi S.H., Silverman R.B., et al. The Sirtuin 2 Inhibitor AK-7 Is Neuroprotective in Huntington’s Disease Mouse Models. Cell Rep. 2012;2:1492-1497.

     Not in our library10.1016/j.celrep.2012.11.001

369. 369.

     Naia L., Carmo C., Campesan S., Fão L., Cotton V.E., Valero J., Lopes C., Rosenstock T.R., Giorgini F., Rego A.C. Mitochondrial SIRT3 confers neuroprotection in Huntington’s disease by regulation of oxidative challenges and mitochondrial dynamics. Free Radic. Biol. Med. 2021;163:163-179.

     Not in our library10.1016/j.freeradbiomed.2020.11.031

370. 370.

     Yeh H.H., Young D., Gelovani J.G., Robinson A., Davidson Y., Herholz K., Mann D.M.A. Histone deacetylase class II and acetylated core histone immunohistochemistry in human brains with Huntington’s disease. Brain Res. 2013;1504:16-24.

     Not in our library10.1016/j.brainres.2013.02.012

371. 371.

     Sharma S., Taliyan R. Transcriptional dysregulation in Huntington’s disease: The role of histone deacetylases. Pharmacol. Res. 2015;100:157-169.

     Not in our library10.1016/j.phrs.2015.08.002

372. 372.

     Bassi S., Tripathi T., Monziani A., Di Leva F., Biagioli M. Epigenetics of huntington’s disease. Advances in Experimental Medicine and Biology. 2017;Volume 978:277-299.

     Not in our library10.1007/978-3-319-53889-1_15

373. 373.

     Sharma S., Sarathlal K.C., Taliyan R. Epigenetics in Neurodegenerative Diseases: The Role of Histone Deacetylases. CNS Neurol. Disord. Drug Targets. 2018;18:11-18.

     Not in our library10.2174/1871527317666181004155136

374. 374.

     Hockly E., Richon V.M., Woodman B., Smith D.L., Zhou X., Rosa E., Sathasivam K., Ghazi-Noori S., Mahal A., Lowden P.A.S., et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington’s disease. Proc. Natl. Acad. Sci. USA. 2003;100:2041-2046.

     Not in our library10.1073/pnas.0437870100

375. 375.

     Ferrante R.J., Kubilus J.K., Lee J., Ryu H., Beesen A., Zucker B., Smith K., Kowall N.W., Ratan R.R., Luthi-Carter R., et al. Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington’s Disease Mice. J. Neurosci. 2003;23:9418-9427.

     Not in our library10.1523/jneurosci.23-28-09418.2003

376. 376.

     Oliveira J.M.A., Chen S., Almeida S., Riley R., Gonçalves J., Oliveira C.R., Hayden M.R., Nicholls D.G., Ellerby L.M., Rego A.C. Mitochondrial-dependent Ca2+ handling in Huntington’s disease striatal cells: Effect of histone deacetylase inhibitors. J. Neurosci. 2006;26:11174-11186.

     Not in our library10.1523/jneurosci.3004-06.2006

377. 377.

     Naia L., Cunha-Oliveira T., Rodrigues J., Rosenstock T.R., Oliveira A., Ribeiro M., Carmo C., Oliveira-Sousa S.I., Duarte A.I., Hayden M.R., et al. Histone deacetylase inhibitors protect against pyruvate dehydrogenase dysfunction in huntington’s disease. J. Neurosci. 2017;37:2776-2794.

     Not in our library10.1523/jneurosci.2006-14.2016

378. 378.

     Thomas E.A., Coppola G., Desplats P.A., Tang B., Soragni E., Burnett R., Gao F., Fitzgerald K.M., Borok J.F., Herman D., et al. The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington’s disease transgenic mice. Proc. Natl. Acad. Sci. USA. 2008;105:15564-15569.

     Not in our library10.1073/pnas.0804249105

379. 379.

     Jia H., Kast R.J., Steffan J.S., Thomas E.A. Selective histone deacetylase (HDAC) inhibition imparts beneficial effects in Huntington’s disease mice: Implications for the ubiquitin-proteasomal and autophagy systems. Hum. Mol. Genet. 2012;21:5280-5293.

     Not in our library10.1093/hmg/dds379

380. 380.

     Jia H., Wang Y., Morris C.D., Jacques V., Gottesfeld J.M., Rusche J.R., Thomas E.A. The effects of pharmacological inhibition of histone deacetylase 3 (HDAC3) in Huntington’s disease mice. PLoS ONE. 2016;11:e0152498.

     Not in our library10.1371/journal.pone.0152498

381. 381.

     Chopra V., Quinti L., Khanna P., Paganetti P., Kuhn R., Young A.B., Kazantsev A.G., Hersch S. LBH589, A Hydroxamic Acid-Derived HDAC Inhibitor, is Neuroprotective in Mouse Models of Huntington’s Disease. J. Huntingtons. Dis. 2016;5:347-355.

     Not in our library10.3233/jhd-160226

382. 382.

     Bobrowska A., Paganetti P., Matthias P., Bates G.P. Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington’s disease. PLoS ONE. 2011;6:e20696.

     Not in our library10.1371/journal.pone.0020696

383. 383.

     Üner M., Yener G. Importance of solid lipid nanoparticles (SLN) in various administration routes and future perspective. Int. J. Nanomed. 2007;2:289-300.

     Not in our library

384. 384.

     Saraiva C., Praça C., Ferreira R., Santos T., Ferreira L., Bernardino L. Nanoparticle-mediated brain drug delivery: Overcoming blood–brain barrier to treat neurodegenerative diseases. J. Control. Release. 2016;235:34-47.

     Not in our library10.1016/j.jconrel.2016.05.044

385. 385.

     Chowdhury A., Kunjiappan S., Panneerselvam T., Somasundaram B., Bhattacharjee C. Nanotechnology and nanocarrier-based approaches on treatment of degenerative diseases. Int. Nano Lett. 2017;7:91-122.

     Not in our library10.1007/s40089-017-0208-0

386. 386.

     Poovaiah N., Davoudi Z., Peng H., Schlichtmann B., Mallapragada S., Narasimhan B., Wang Q. Treatment of neurodegenerative disorders through the blood–brain barrier using nanocarriers. Nanoscale. 2018;10:16962-16983.

     Not in our library10.1039/c8nr04073g

387. 387.

     Rakotoarisoa M., Angelova A. Amphiphilic Nanocarrier Systems for Curcumin Delivery in Neurodegenerative Disorders. Medicines. 2018;5:126.

     Not in our library10.3390/medicines5040126

388. 388.

     Wilson E.R., Parker L.M., Orth A., Nunn N., Torelli M., Shenderova O., Gibson B.C., Reineck P. The effect of particle size on nanodiamond fluorescence and colloidal properties in biological media. Nanotechnology. 2019;30:385704.

     Not in our library10.1088/1361-6528/ab283d

389. 389.

     Ramachandran S., Thangarajan S. A novel therapeutic application of solid lipid nanoparticles encapsulated thymoquinone (TQ-SLNs) on 3-nitroproponic acid induced Huntington’s disease-like symptoms in wistar rats. Chem. Biol. Interact. 2016;256:25-36.

     Not in our library10.1016/j.cbi.2016.05.020

390. 390.

     Ramachandran S., Thangarajan S. Thymoquinone loaded solid lipid nanoparticles counteracts 3-Nitropropionic acid induced motor impairments and neuroinflammation in rat model of Huntington’s disease. Metab. Brain Dis. 2018;33:1459-1470.

     Not in our library10.1007/s11011-018-0252-0

391. 391.

     Bhatt R., Singh D., Prakash A., Mishra N. Development, characterization and nasal delivery of rosmarinic acid-loaded solid lipid nanoparticles for the effective management of Huntingtons disease. Drug Deliv. 2015;22:931-939.

     Not in our library10.3109/10717544.2014.880860

392. 392.

     Niu X., Chen J., Gao J. Nanocarriers as a powerful vehicle to overcome blood-brain barrier in treating neurodegenerative diseases: Focus on recent advances. Asian J. Pharm. Sci. 2019;14:480-496.

     Not in our library10.1016/j.ajps.2018.09.005

393. 393.

     Bahmad H., Hadadeh O., Chamaa F., Cheaito K., Darwish B., Makkawi A.-K., Abou-Kheir W. Modeling Human Neurological and Neurodegenerative Diseases: From Induced Pluripotent Stem Cells to Neuronal Differentiation and Its Applications in Neurotrauma. Front. Mol. Neurosci. 2017;10:1-17.

     Not in our library10.3389/fnmol.2017.00050

394. 394.

     Connor B. Concise Review: The Use of Stem Cells for Understanding and Treating Huntington’s Disease. Stem Cells. 2018;36:146-160.

     Not in our library10.1002/stem.2747

395. 395.

     Holley S.M., Kamdjou T., Reidling J.C., Fury B., Coleal-Bergum D., Bauer G., Thompson L.M., Levine M.S., Cepeda C. Therapeutic effects of stem cells in rodent models of Huntington’s disease: Review and electrophysiological findings. CNS Neurosci. Ther. 2018;24:329-342.

     Not in our library10.1111/cns.12839

396. 396.

     Kerkis I., Haddad M.S., Valverde C.W., Glosman S. Neural and mesenchymal stem cells in animal models of Huntington’s disease: Past experiences and future challenges. Stem Cell Res. Ther. 2015;6:232.

     Not in our library10.1186/s13287-015-0248-1

397. 397.

     Lo Furno D., Mannino G., Giuffrida R. Functional role of mesenchymal stem cells in the treatment of chronic neurodegenerative diseases. J. Cell. Physiol. 2018;233:3982-3999.

     Not in our library10.1002/jcp.26192

398. 398.

     Golas M.M. Human cellular models of medium spiny neuron development and Huntington disease. Life Sci. 2018;209:179-196.

     Not in our library10.1016/j.lfs.2018.07.030

399. 399.

     Colpo G.D., Furr Stimming E., Teixeira A.L. Stem cells in animal models of Huntington disease: A systematic review. Mol. Cell. Neurosci. 2019;95:43-50.

     Not in our library10.1016/j.mcn.2019.01.006

400. 400.

     Dunnett S.B., Carter R.J., Watts C., Torres E.M., Mahal A., Mangiarini L., Bates G., Morton A.J. Striatal Transplantation in a Transgenic Mouse Model of Huntington’s Disease. Exp. Neurol. 1998;154:31-40.

     Not in our library10.1006/exnr.1998.6926

401. 401.

     van Dellen A., Deacon R., York D., Blakemore C., Hannan A.J. Anterior cingulate cortical transplantation in transgenic Huntington’s disease mice. Brain Res. Bull. 2001;56:313-318.

     Not in our library10.1016/s0361-9230(01)00655-4

402. 402.

     Snyder E.Y., Yoon C., Flax J.D., Macklis J.D. Multipotent neural precursors can differentiate toward replacement of neurons undergoing targeted apoptotic degeneration in adult mouse neocortex. Proc. Natl. Acad. Sci. USA. 1997;94:11663-11668.

     Not in our library10.1073/pnas.94.21.11663

403. 403.

     Lee J.P., Jeyakumar M., Gonzalez R., Takahashi H., Lee P.J., Baek R.C., Clark D., Rose H., Fu G., Clarke J., et al. Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. Nat. Med. 2007;13:439-447.

     Not in our library10.1038/nm1548

404. 404.

     Yang C.R., Yu R.K. Intracerebral transplantation of neural stem cells combined with trehalose ingestion alleviates pathology in a mouse model of Huntington’s disease. J. Neurosci. Res. 2009;87:26-33.

     Not in our library10.1002/jnr.21817

405. 405.

     Fink K.D., Rossignol J., Crane A.T., Davis K.K., Bombard M.C., Bavar A.M., Clerc S., Lowrance S.A., Song C., Lescaudron L., et al. Transplantation of umbilical cord-derived mesenchymal stem cells into the striata of R6/2 mice: Behavioral and neuropathological analysis. Stem Cell Res. Ther. 2013;4:130.

     Not in our library10.1186/scrt341

406. 406.

     Rossignol J., Fink K.D., Crane A.T., Davis K.K., Bombard M.C., Clerc S., Bavar A.M., Lowrance S.A., Song C., Witte S., et al. Reductions in behavioral deficits and neuropathology in the R6/2 mouse model of Huntington’s disease following transplantation of bone-marrow-derived mesenchymal stem cells is dependent on passage number. Stem Cell Res. Ther. 2015;6:9.

     Not in our library10.1186/scrt545

407. 407.

     Giampà C., Alvino A., Magatti M., Silini A.R., Cardinale A., Paldino E., Fusco F.R., Parolini O. Conditioned medium from amniotic cells protects striatal degeneration and ameliorates motor deficits in the R6/2 mouse model of Huntington’s disease. J. Cell. Mol. Med. 2019;23:1581-1592.

     Not in our library10.1111/jcmm.14113

408. 408.

     Reidling J.C., Relaño-Ginés A., Holley S.M., Ochaba J., Moore C., Fury B., Lau A., Tran A.H., Yeung S., Salamati D., et al. Human Neural Stem Cell Transplantation Rescues Functional Deficits in R6/2 and Q140 Huntington’s Disease Mice. Stem Cell Reports. 2018;10:58-72.

     Not in our library10.1016/j.stemcr.2017.11.005

409. 409.

     Dey N.D., Bombard M.C., Roland B.P., Davidson S., Lu M., Rossignol J., Sandstrom M.I., Skeel R.L., Lescaudron L., Dunbar G.L. Genetically engineered mesenchymal stem cells reduce behavioral deficits in the YAC 128 mouse model of Huntington’s disease. Behav. Brain Res. 2010;214:193-200.

     Not in our library10.1016/j.bbr.2010.05.023

410. 410.

     Al-Gharaibeh A., Culver R., Stewart A.N., Srinageshwar B., Spelde K., Frollo L., Kolli N., Story D., Paladugu L., Anwar S., et al. Induced pluripotent stem cell-derived neural stem cell transplantations reduced behavioral deficits and ameliorated neuropathological changes in YAC128 mouse model of Huntington’s disease. Front. Neurosci. 2017;11:628.

     Not in our library10.3389/fnins.2017.00628

411. 411.

     Wang Y.L., Liu W., Wada E., Murata M., Wada K., Kanazawa I. Clinico-pathological rescue of a model mouse of Huntington’s disease by siRNA. Neurosci. Res. 2005;53:241-249.

     Not in our library10.1016/j.neures.2005.06.021

412. 412.

     DiFiglia M., Sena-Esteves M., Chase K., Sapp E., Pfister E., Sass M., Yoder J., Reeves P., Pandey R.K., Rajeev K.G., et al. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc. Natl. Acad. Sci. USA. 2007;104:17204-17209.

     Not in our library10.1073/pnas.0708285104

413. 413.

     Bilsen P.H.J.V., Jaspers L., Lombardi M.S., Odekerken J.C.E., Burright E.N., Kaemmerer W.F. Identification and allele-specific silencing of the mutant huntingtin allele in Huntington’s disease patient-derived fibroblasts. Hum. Gene Ther. 2008;19:710-718.

     Not in our library10.1089/hum.2007.116

414. 414.

     Lombardi M.S., Jaspers L., Spronkmans C., Gellera C., Taroni F., Di Maria E., Di Donato S., Kaemmerer W.F. A majority of Huntington’s disease patients may be treatable by individualized allele-specific RNA interference. Exp. Neurol. 2009;217:312-319.

     Not in our library10.1016/j.expneurol.2009.03.004

415. 415.

     Jimenez-Sanchez M., Lam W., Hannus M., Sönnichsen B., Imarisio S., Fleming A., Tarditi A., Menzies F., Ed Dami T., Xu C., et al. SiRNA screen identifies QPCT as a druggable target for Huntington’s disease. Nat. Chem. Biol. 2015;11:347-354.

     Not in our library10.1038/nchembio.1790

416. 416.

     Ban J.J., Chung J.Y., Lee M., Im W., Kim M. MicroRNA-27a reduces mutant hutingtin aggregation in an in vitro model of Huntington’s disease. Biochem. Biophys. Res. Commun. 2017;488:316-321.

     Not in our library10.1016/j.bbrc.2017.05.040

417. 417.
     Allele-specific silencing of mutant huntingtin in rodent brain and human stem cells. · 2014

     Not checked
418. 418.

     Boudreau R.L., McBride J.L., Martins I., Shen S., Xing Y., Carter B.J., Davidson B.L. Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington’s disease mice. Mol. Ther. 2009;17:1053-1063.

     Not in our library10.1038/mt.2009.17

419. 419.

     McBride J.L., Pitzer M.R., Boudreau R.L., Dufour B., Hobbs T., Ojeda S.R., Davidson B.L. Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington’s disease. Mol. Ther. 2011;19:2152-2162.

     Not in our library10.1038/mt.2011.219

420. 420.

     Huang B., Schiefer J., Sass C., Landwehrmeyer G.B., Kosinski C.M., Kochanek S. High-capacity adenoviral vector-mediated reduction of huntingtin aggregate load in vitro and in vivo. Hum. Gene Ther. 2007;18:303-311.

     Not in our library10.1089/hum.2006.160

421. 421.

     Grimm D., Streetz K.L., Jopling C.L., Storm T.A., Pandey K., Davis C.R., Marion P., Salazar F., Kay M.A. Fatality in mice due to oversaturation of cellular microRNA/short hairpin RNA pathways. Nature. 2006;441:537-541.

     Not in our library10.1038/nature04791

422. 422.

     McBride J.L., Boudreau R.L., Harper S.Q., Staber P.D., Monteys A.M., Martins I., Gilmore B.L., Burstein H., Peluso R.W., Polisky B. Artificial miRNAs mitigate shRNA-mediated toxicity in the brain: Implications for the therapeutic development of RNAi. Proc. Natl. Acad. Sci. USA. 2008;105:5868-5873.

     Not in our library10.1073/pnas.0801775105

423. 423.

     Aguiar S., van der Gaag B., Cortese F.A.B. RNAi mechanisms in Huntington’s disease therapy: siRNA versus shRNA. Transl. Neurodegener. 2017;6:30.

     Not in our library10.1186/s40035-017-0101-9

424. 424.

     Keeler A.M., Sapp E., Chase K., Sottosanti E., Danielson E., Pfister E., Stoica L., Difiglia M., Aronin N., Sena-Esteves M. Cellular Analysis of Silencing the Huntington’s Disease Gene Using AAV9 Mediated Delivery of Artificial Micro RNA into the Striatum of Q140/Q140 Mice. J. Huntingt. Dis. 2016;5:239-248.

     Not in our library10.3233/jhd-160215

425. 425.

     Pfister E.L., Chase K.O., Sun H., Kennington L.A., Conroy F., Johnson E., Miller R., Borel F., Aronin N., Mueller C. Safe and efficient silencing with a Pol II, but not a Pol lII, promoter expressing an artificial miRNA targeting human huntingtin. Mol. Ther. Nucleic Acids. 2017;7:324-334.

     Not in our library10.1016/j.omtn.2017.04.011

426. 426.

     Jinek M., Chylinski K., Fonfara I., Hauer M., Doudna J.A., Charpentier E. A programmable dual-RNA-guided DNA endonuclease in adaptive bacterial immunity. Science. 2012;337:816-821.

     Not in our library10.1126/science.1225829

427. 427.

     Shin J.W., Kim K.H., Chao M.J., Atwal R.S., Gillis T., MacDonald M.E., Gusella J.F., Lee J.M. Permanent inactivation of Huntington’s disease mutation by personalized allele-specific CRISPR/Cas9. Hum. Mol. Genet. 2016;25:4566-4576.

     Not in our library10.1093/hmg/ddw286

428. 428.

     Dabrowska M., Juzwa W., Krzyzosiak W.J., Olejniczak M. Precise excision of the CAG tract from the huntingtin gene by Cas9 nickases. Front. Neurosci. 2018;12:75.

     Not in our library10.3389/fnins.2018.00075

429. 429.

     Yang S., Chang R., Yang H., Zhao T., Hong Y., Kong H.E., Sun X., Qin Z., Jin P., Li S., et al. CRISPR/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington’s disease. J. Clin. Investig. 2017;127:2719-2724.

     Not in our library10.1172/jci92087

430. 430.
     The Self-Inactivating KamiCas9 System for the Editing of CNS Disease Genes · 2017

     Not checked
431. 431.

     Kosicki M., Tomberg K., Bradley A. Repair of double-strand breaks induced by CRISPR–Cas9 leads to large deletions and complex rearrangements. Nat. Biotechnol. 2018;36:765-771.

     Not in our library10.1038/nbt.4192

432. 432.

     Tabrizi S.J., Ghosh R., Leavitt B.R. Huntingtin Lowering Strategies for Disease Modification in Huntington’s Disease. Neuron. 2019;102:801-819.

     Not in our library10.1016/j.neuron.2019.01.039

433. 433.

     Sledzinski P., Dabrowska M., Nowaczyk M., Olejniczak M. Paving the way towards precise and safe CRISPR genome editing. Biotechnol. Adv. 2021;49:107737.

     Not in our library10.1016/j.biotechadv.2021.107737

434. 434.

     Li J.Y., Popovic N., Brundin P. The use of the R6 transgenic mouse models of Huntington’ s disease in attempts to develop novel therapeutic strategies. NeuroRx. 2005;2:447-464.

     Not in our library10.1602/neurorx.2.3.447

435. 435.

     Olanow C.W., Wunderle K.B., Kieburtz K. Milestones in movement disorders clinical trials: Advances and landmark studies. Mov. Disord. 2011;26:1003-1014.

     Not in our library10.1002/mds.23727

436. 436.

     Ravina B., Romer M., Constantinescu R., Biglan K., Brocht A., Kieburtz K., Shoulson I., McDermott M.P. The relationship between CAG repeat length and clinical progression in Huntington’s disease. Mov. Disord. 2008;23:1223-1227.

     Not in our library10.1002/mds.21988

437. 437.

     Stout J.C., Jones R., Labuschagne I., O’Regan A.M., Say M.J., Dumas E.M., Queller S., Justo D., Dar Santos R., Coleman A., et al. Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington’s disease. J. Neurol. Neurosurg. Psychiatry. 2012;83:687-694.

     Not in our library10.1136/jnnp-2011-301940

Cited by papers we hold 2

• The role of neuroinflammation in neurodegenerative diseases: current understanding and future therapeutic targets · 2024
  No notice
• Enriched Environment and Exercise Enhance Stem Cell Therapy for Stroke, Parkinson’s Disease, and Huntington’s Disease · 2022
  Not checked

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