-
1
-
-
78650031174
-
Huntington's disease: From molecular pathogenesis to clinical treatment
-
Ross CA, Tabrizi SJ. Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol. 2011;10(1):83-98.
-
(2011)
Lancet Neurol
, vol.10
, Issue.1
, pp. 83-98
-
-
Ross, C.A.1
Tabrizi, S.J.2
-
2
-
-
84904065460
-
An update on Huntington's disease: From the gene to the clinic
-
Kim SD, Fung VS. An update on Huntington's disease: from the gene to the clinic. Curr Opin Neurol. 2014;27(4):477-483.
-
(2014)
Curr Opin Neurol
, vol.27
, Issue.4
, pp. 477-483
-
-
Kim, S.D.1
Fung, V.S.2
-
3
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group
-
[No authors listed]
-
[No authors listed]. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell. 1993;72(6): 971-983.
-
(1993)
Cell
, vol.72
, Issue.6
, pp. 971-983
-
-
-
4
-
-
84904580025
-
Investigating the structural impact of the glutamine repeat in huntingtin assembly
-
107
-
Perevozchikova T, Stanley CB, McWilliams-Koeppen HP, Rowe EL, Berthelier V. Investigating the structural impact of the glutamine repeat in huntingtin assembly. Biophys J. 15, 2014;107(2):411-421.
-
(2014)
Biophys J
, vol.15
, Issue.2
, pp. 411-421
-
-
Perevozchikova, T.1
Stanley, C.B.2
McWilliams-Koeppen, H.P.3
Rowe, E.L.4
Berthelier, V.5
-
5
-
-
79551554590
-
The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease
-
Munoz-Sanjuan I, Bates GP. The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease. J Clin Invest. 2011;121(2):476-483.
-
(2011)
J Clin Invest
, vol.121
, Issue.2
, pp. 476-483
-
-
Munoz-Sanjuan, I.1
Bates, G.P.2
-
6
-
-
84896790519
-
A large scale huntingtin protein interaction network implicates Rho GTPase signaling pathways in Huntington disease
-
Tourette C, Li B, Bell R, et al. A large scale huntingtin protein interaction network implicates Rho GTPase signaling pathways in Huntington disease. J Biol Chem. 2014;289(10):6709-6726.
-
(2014)
J Biol Chem
, vol.289
, Issue.10
, pp. 6709-6726
-
-
Tourette, C.1
Li, B.2
Bell, R.3
-
7
-
-
84861402086
-
Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis
-
Ratovitski T, Chighladze E, Arbez N, et al. Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis. Cell Cycle. 2012;11(10):2006-2021.
-
(2012)
Cell Cycle
, vol.11
, Issue.10
, pp. 2006-2021
-
-
Ratovitski, T.1
Chighladze, E.2
Arbez, N.3
-
8
-
-
84874451388
-
Recent advances in the management of choreas
-
Burgunder JM. Recent advances in the management of choreas. Ther Adv Neurol Dis. 2013;6(2):117-127.
-
(2013)
Ther Adv Neurol Dis
, vol.6
, Issue.2
, pp. 117-127
-
-
Burgunder, J.M.1
-
9
-
-
68249113963
-
Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: Cross-sectional analysis of baseline data
-
Tabrizi SJ, Langbehn DR, Leavitt BR, et al. Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol. 2009;8(9): 791-801.
-
(2009)
Lancet Neurol
, vol.8
, Issue.9
, pp. 791-801
-
-
Tabrizi, S.J.1
Langbehn, D.R.2
Leavitt, B.R.3
-
10
-
-
78650026297
-
Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: The 12-month longitudinal analysis
-
Tabrizi SJ, Scahill RI, Durr A, et al. Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol. 2011;10(1):31-42.
-
(2011)
Lancet Neurol
, vol.10
, Issue.1
, pp. 31-42
-
-
Tabrizi, S.J.1
Scahill, R.I.2
Durr, A.3
-
11
-
-
83555173501
-
Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: Analysis of 24 month observational data
-
Tabrizi SJ, Reilmann R, Roos RA, et al. Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol. 2012;11(1):42-53.
-
(2012)
Lancet Neurol
, vol.11
, Issue.1
, pp. 42-53
-
-
Tabrizi, S.J.1
Reilmann, R.2
Roos, R.A.3
-
12
-
-
20244378556
-
RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
-
Harper SQ, Staber PD, He X, et al. RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proc Natl Acad Sci U S A. 2005;102(16):5820-5825.
-
(2005)
Proc Natl Acad Sci U S A
, vol.102
, Issue.16
, pp. 5820-5825
-
-
Harper, S.Q.1
Staber, P.D.2
He, X.3
-
13
-
-
67349100160
-
Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice
-
Boudreau RL, McBride JL, Martins I, et al. Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice. Mol Ther. 2009;17(6):1053-1063.
-
(2009)
Mol Ther
, vol.17
, Issue.6
, pp. 1053-1063
-
-
Boudreau, R.L.1
McBride, J.L.2
Martins, I.3
-
14
-
-
79551530731
-
Oligonucleotide therapeutic approaches for Huntington disease
-
Sah DW, Aronin N. Oligonucleotide therapeutic approaches for Huntington disease. J Clin Invest. 2011;121(2):500-507.
-
(2011)
J Clin Invest
, vol.121
, Issue.2
, pp. 500-507
-
-
Sah, D.W.1
Aronin, N.2
-
15
-
-
84862663712
-
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis
-
Kordasiewicz HB, Stanek LM, Wancewicz EV, et al. Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron. 2012;74(6):1031-1044.
-
(2012)
Neuron
, vol.74
, Issue.6
, pp. 1031-1044
-
-
Kordasiewicz, H.B.1
Stanek, L.M.2
Wancewicz, E.V.3
-
16
-
-
36749033738
-
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
-
DiFiglia M, Sena-Esteves M, Chase K, et al. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc Natl Acad Sci U S A. 2007;104(43): 17204-17209.
-
(2007)
Proc Natl Acad Sci U S A
, vol.104
, Issue.43
, pp. 17204-17209
-
-
DiFiglia, M.1
Sena-Esteves, M.2
Chase, K.3
-
17
-
-
67349159137
-
Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients
-
Pfister EL, Kennington L, Straubhaar J, et al. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol. 2009;19(9):774-778.
-
(2009)
Curr Biol
, vol.19
, Issue.9
, pp. 774-778
-
-
Pfister, E.L.1
Kennington, L.2
Straubhaar, J.3
-
18
-
-
77952688315
-
A randomized, double-blind, placebo-controlled study of an RNAi-based therapy directed against respiratory syncytial virus
-
DeVincenzo J, Lambkin-Williams R, Wilkinson T, et al. A randomized, double-blind, placebo-controlled study of an RNAi-based therapy directed against respiratory syncytial virus. Proc Natl Acad Sci U S A. 2010;107(19):8800-8805.
-
(2010)
Proc Natl Acad Sci U S A
, vol.107
, Issue.19
, pp. 8800-8805
-
-
DeVincenzo, J.1
Lambkin-Williams, R.2
Wilkinson, T.3
-
19
-
-
84860192454
-
Six-month partial suppression of huntingtin is well tolerated in the adult rhesus striatum
-
Grondin R, Kaytor MD, Ai Y, et al. Six-month partial suppression of huntingtin is well tolerated in the adult rhesus striatum. Brain. 2012;135(Pt 4):1197-1209.
-
(2012)
Brain
, vol.135
, pp. 1197-1209
-
-
Grondin, R.1
Kaytor, M.D.2
Ai, Y.3
-
20
-
-
84869883424
-
Therapeutic approaches to preventing cell death in Huntington disease
-
Kaplan A, Stockwell BR. Therapeutic approaches to preventing cell death in Huntington disease. Prog Neurobiol. 2012;99(3):262-280.
-
(2012)
Prog Neurobiol
, vol.99
, Issue.3
, pp. 262-280
-
-
Kaplan, A.1
Stockwell, B.R.2
-
21
-
-
84865688581
-
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression
-
Yu D, Pendergraff H, Liu J, et al. Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell. 2012;150(5):895-908.
-
(2012)
Cell
, vol.150
, Issue.5
, pp. 895-908
-
-
Yu, D.1
Pendergraff, H.2
Liu, J.3
-
22
-
-
84901688904
-
Personalized gene silencing therapeutics for Huntington disease
-
Kay C, Skotte NH, Southwell AL, Hayden MR. Personalized gene silencing therapeutics for Huntington disease. Clin Genet. 2014;86(1): 29-36.
-
(2014)
Clin Genet
, vol.86
, Issue.1
, pp. 29-36
-
-
Kay, C.1
Skotte, N.H.2
Southwell, A.L.3
Hayden, M.R.4
-
23
-
-
32044458625
-
Designer zinc-finger proteins and their applications
-
Papworth M, Kolasinska P, Minczuk M. Designer zinc-finger proteins and their applications. Gene. 2006;366(1):27-38.
-
(2006)
Gene
, vol.366
, Issue.1
, pp. 27-38
-
-
Papworth, M.1
Kolasinska, P.2
Minczuk, M.3
-
24
-
-
84868538087
-
Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice
-
Garriga-Canut M, Agustín-Pavón C, Herrmann F, et al. Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice. Proc Natl Acad Sci U S A. 2012;109(45):E3136-E3145.
-
(2012)
Proc Natl Acad Sci U S A
, vol.109
, Issue.45
, pp. E3136-E3145
-
-
Garriga-Canut, M.1
Agustín-Pavón, C.2
Herrmann, F.3
-
25
-
-
80053573543
-
Small changes, big impact: Posttranslational modifications and function of huntingtin in Huntington disease
-
Ehrnhoefer DE, Sutton L, Hayden MR. Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease. Neuroscientist. 2011;17(5):475-492.
-
(2011)
Neuroscientist
, vol.17
, Issue.5
, pp. 475-492
-
-
Ehrnhoefer, D.E.1
Sutton, L.2
Hayden, M.R.3
-
26
-
-
33748744375
-
Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models
-
Ehrnhoefer DE, Duennwald M, Markovic P, et al. Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models. Hum Mol Genet. 2006;15(18):2743-2751.
-
(2006)
Hum Mol Genet
, vol.15
, Issue.18
, pp. 2743-2751
-
-
Ehrnhoefer, D.E.1
Duennwald, M.2
Markovic, P.3
-
27
-
-
84899965665
-
A potent and selective sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease
-
Smith MR, Syed A, Lukacsovich T, 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(11):2995-3007.
-
(2014)
Hum Mol Genet
, vol.23
, Issue.11
, pp. 2995-3007
-
-
Smith, M.R.1
Syed, A.2
Lukacsovich, T.3
-
28
-
-
84883553202
-
A randomised, double-blind, placebo-controlled phase IB pharmacodynamic study with selisistat (SEN0014196) in HD patients
-
Süssmuth S, Landwehrmeyer G, Tabrizi S, et al. A randomised, double-blind, placebo-controlled phase IB pharmacodynamic study with selisistat (SEN0014196) in HD patients. J Neurol Neurosurg Psychiatry. 2012;83(Suppl 1):A55.
-
(2012)
J Neurol Neurosurg Psychiatry
, vol.83
-
-
Süssmuth, S.1
Landwehrmeyer, G.2
Tabrizi, S.3
-
29
-
-
84907928725
-
Safety and tolerability of selisistat for the treatment of Huntington's disease: Results from a randomized, double-blind, placebo-controlled phase II trial
-
Reilmann R, Squitieri F, Priller J, et al. Safety and tolerability of selisistat for the treatment of Huntington's disease: results from a randomized, double-blind, placebo-controlled phase II trial. Neurology. 2014;82(Suppl 10):S47004.
-
(2014)
Neurology
, vol.82
-
-
Reilmann, R.1
Squitieri, F.2
Priller, J.3
-
30
-
-
84860123776
-
Suppression of protein aggregation by chaperone modification of high molecular weight complexes
-
Labbadia J, Novoselov SS, Bett JS, et al. Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain. 2012;135(Pt 4):1180-1196.
-
(2012)
Brain
, vol.135
, pp. 1180-1196
-
-
Labbadia, J.1
Novoselov, S.S.2
Bett, J.S.3
-
31
-
-
84860692601
-
Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins
-
Butler DC, McLear JA, Messer A. Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins. Prog Neurobiol. 2012;97(2):190-204.
-
(2012)
Prog Neurobiol
, vol.97
, Issue.2
, pp. 190-204
-
-
Butler, D.C.1
McLear, J.A.2
Messer, A.3
-
32
-
-
0035127907
-
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
-
Leavitt BR, Guttman JA, Hodgson JG, et al. Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. Am J Hum Genet. 2001;68(2):313-324.
-
(2001)
Am J Hum Genet
, vol.68
, Issue.2
, pp. 313-324
-
-
Leavitt, B.R.1
Guttman, J.A.2
Hodgson, J.G.3
-
33
-
-
54949140807
-
Protective role of engrailed in a Drosophila model of Huntington's disease
-
Mugat B, Parmentier ML, Bonneaud N, Chan HY, Maschat F. Protective role of engrailed in a Drosophila model of Huntington's disease. Hum Mol Genet. 2008;17(22):3601-3616.
-
(2008)
Hum Mol Genet
, vol.17
, Issue.22
, pp. 3601-3616
-
-
Mugat, B.1
Parmentier, M.L.2
Bonneaud, N.3
Chan, H.Y.4
Maschat, F.5
-
34
-
-
84936787935
-
Systemic delivery of P42 peptide to fight Huntington's disease
-
Bonneaud N, Paucard A, Bauer C, et al. Systemic delivery of P42 peptide to fight Huntington's disease. J Neurol Neurosurg Psych. 2014;85(Suppl 1):A98.
-
(2014)
J Neurol Neurosurg Psych
, vol.85
-
-
Bonneaud, N.1
Paucard, A.2
Bauer, C.3
-
36
-
-
33750314788
-
Immunohistochemical localization of phosphodiesterase 10A in multiple mammalian species
-
Coskran TM, Morton D, Menniti FS, et al. Immunohistochemical localization of phosphodiesterase 10A in multiple mammalian species. J Histochem Cytochem. 2006;54(11):1205-1213.
-
(2006)
J Histochem Cytochem
, vol.54
, Issue.11
, pp. 1205-1213
-
-
Coskran, T.M.1
Morton, D.2
Menniti, F.S.3
-
37
-
-
0037335074
-
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice
-
Gines S, Seong IS, Fossale E, et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet. 2003;12(5):497-508.
-
(2003)
Hum Mol Genet
, vol.12
, Issue.5
, pp. 497-508
-
-
Gines, S.1
Seong, I.S.2
Fossale, E.3
-
38
-
-
42449099422
-
Preclinical characterization of selective phosphodiesterase 10A inhibitors: A new therapeutic approach to the treatment of schizophrenia
-
Schmidt CJ, Chapin DS, Cianfrogna J, et al. Preclinical characterization of selective phosphodiesterase 10A inhibitors: a new therapeutic approach to the treatment of schizophrenia. J Pharmacol Exp Ther. 2008;325(2):681-690.
-
(2008)
J Pharmacol Exp Ther
, vol.325
, Issue.2
, pp. 681-690
-
-
Schmidt, C.J.1
Chapin, D.S.2
Cianfrogna, J.3
-
39
-
-
70350445535
-
Phosphodiesterase 10A inhibitor activity in preclinical models of the positive, cognitive, and negative symptoms of schizophrenia
-
Grauer SM, Pulito VL, Navarra RL, et al. Phosphodiesterase 10A inhibitor activity in preclinical models of the positive, cognitive, and negative symptoms of schizophrenia. J Pharmacol Exp Ther. 2009;331(2):574-590.
-
(2009)
J Pharmacol Exp Ther
, vol.331
, Issue.2
, pp. 574-590
-
-
Grauer, S.M.1
Pulito, V.L.2
Navarra, R.L.3
-
40
-
-
33745958765
-
Inhibition of the striatum-enriched phosphodiesterase PDE10A: A novel approach to the treatment of psychosis
-
Siuciak JA, Chapin DS, Harms JF, et al. Inhibition of the striatum-enriched phosphodiesterase PDE10A: a novel approach to the treatment of psychosis. Neuropharmacology. 2006;51(2):386-396.
-
(2006)
Neuropharmacology
, vol.51
, Issue.2
, pp. 386-396
-
-
Siuciak, J.A.1
Chapin, D.S.2
Harms, J.F.3
-
41
-
-
12944263711
-
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
-
Steffan JS, Kazantsev A, Spasic-Boskovic O, et al. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000;97(12):6763-6768.
-
(2000)
Proc Natl Acad Sci U S A
, vol.97
, Issue.12
, pp. 6763-6768
-
-
Steffan, J.S.1
Kazantsev, A.2
Spasic-Boskovic, O.3
-
42
-
-
78650779872
-
Chronic suppression of phosphodiesterase 10A alters striatal expression of genes responsible for neurotransmitter synthesis, neurotransmission, and signaling pathways implicated in Huntington's disease
-
Kleiman RJ, Kimmel LH, Bove SE, et al. Chronic suppression of phosphodiesterase 10A alters striatal expression of genes responsible for neurotransmitter synthesis, neurotransmission, and signaling pathways implicated in Huntington's disease. J Pharmacol Exp Ther. 2011;336(1):64-76.
-
(2011)
J Pharmacol Exp Ther
, vol.336
, Issue.1
, pp. 64-76
-
-
Kleiman, R.J.1
Kimmel, L.H.2
Bove, S.E.3
-
43
-
-
33645893580
-
Genetic deletion of the striatum-enriched phosphodiesterase PDE10A: Evidence for altered striatal function
-
Siuciak JA, McCarthy SA, Chapin DS, et al. Genetic deletion of the striatum-enriched phosphodiesterase PDE10A: evidence for altered striatal function. Neuropharmacology. 2006;51(2):374-385.
-
(2006)
Neuropharmacology
, vol.51
, Issue.2
, pp. 374-385
-
-
Siuciak, J.A.1
McCarthy, S.A.2
Chapin, D.S.3
-
44
-
-
67349165136
-
Phosphodiesterase 10 inhibition reduces striatal excitotoxicity in the quinolinic acid model of Huntington's disease
-
Giampa C, Patassini S, Borreca A, et al. Phosphodiesterase 10 inhibition reduces striatal excitotoxicity in the quinolinic acid model of Huntington's disease. Neurobiol Dis. 2009;34(3):450-456.
-
(2009)
Neurobiol Dis
, vol.34
, Issue.3
, pp. 450-456
-
-
Giampa, C.1
Patassini, S.2
Borreca, A.3
-
45
-
-
78149452347
-
Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease
-
Giampa C, Laurenti D, Anzilotti S, Bernardi G, Menniti FS, Fusco FR. Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease. PLoS One. 2010;5(10):e13417.
-
(2010)
PLoS One
, vol.5
, Issue.10
-
-
Giampa, C.1
Laurenti, D.2
Anzilotti, S.3
Bernardi, G.4
Menniti, F.S.5
Fusco, F.R.6
-
46
-
-
84880788214
-
PDE10 inhibition increases GluA1 and CREB phosphorylation and improves spatial and recognition memories in a Huntington's disease mouse model
-
Giralt A, Saavedra A, Carreton O, et al. PDE10 inhibition increases GluA1 and CREB phosphorylation and improves spatial and recognition memories in a Huntington's disease mouse model. Hippocampus. 2013;23(8):684-695.
-
(2013)
Hippocampus
, vol.23
, Issue.8
, pp. 684-695
-
-
Giralt, A.1
Saavedra, A.2
Carreton, O.3
-
47
-
-
1642441418
-
Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms
-
Hebb AL, Robertson HA, Denovan-Wright EM. Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms. Neuroscience. 2004;123(4):967-981.
-
(2004)
Neuroscience
, vol.123
, Issue.4
, pp. 967-981
-
-
Hebb, A.L.1
Robertson, H.A.2
Denovan-Wright, E.M.3
-
48
-
-
84895727405
-
PET imaging shows loss of striatal PDE10A in patients with Huntington disease
-
Ahmad R, Bourgeois S, Postnov A, et al. PET imaging shows loss of striatal PDE10A in patients with Huntington disease. Neurology. 2014;82(3):279-281.
-
(2014)
Neurology
, vol.82
, Issue.3
, pp. 279-281
-
-
Ahmad, R.1
Bourgeois, S.2
Postnov, A.3
-
49
-
-
33745392939
-
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin
-
Benchoua A, Trioulier Y, Zala D, et al. Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell. 2006;17(4):1652-1663.
-
(2006)
Mol Biol Cell
, vol.17
, Issue.4
, pp. 1652-1663
-
-
Benchoua, A.1
Trioulier, Y.2
Zala, D.3
-
50
-
-
0030919567
-
Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia
-
Browne SE, Bowling AC, MacGarvey U, et al. Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann Neurol. 1997;41(5):646-653.
-
(1997)
Ann Neurol
, vol.41
, Issue.5
, pp. 646-653
-
-
Browne, S.E.1
Bowling, A.C.2
McGarvey, U.3
-
51
-
-
77957742105
-
Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease
-
Kim J, Moody JP, Edgerly CK, et al. Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease. Hum Mol Genet. 2010;19(20):3919-3935.
-
(2010)
Hum Mol Genet
, vol.19
, Issue.20
, pp. 3919-3935
-
-
Kim, J.1
Moody, J.P.2
Edgerly, C.K.3
-
52
-
-
84859904873
-
Shaping the role of mitochondria in the pathogenesis of Huntington's disease
-
Costa V, Scorrano L. Shaping the role of mitochondria in the pathogenesis of Huntington's disease. EMBO J. 2012;31(8):1853-1864.
-
(2012)
EMBO J
, vol.31
, Issue.8
, pp. 1853-1864
-
-
Costa, V.1
Scorrano, L.2
-
53
-
-
77956802394
-
Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects
-
Hyson HC, Kieburtz K, Shoulson I, et al. Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects. Mov Disord. 2010;25(12):1924-1928.
-
(2010)
Mov Disord
, vol.25
, Issue.12
, pp. 1924-1928
-
-
Hyson, H.C.1
Kieburtz, K.2
Shoulson, I.3
-
54
-
-
84859100749
-
Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in an ALS mouse model
-
Sunyach C, Michaud M, Arnoux T, et al. Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in an ALS mouse model. Neuropharmacology. 2012;62(7):2346-2352.
-
(2012)
Neuropharmacology
, vol.62
, Issue.7
, pp. 2346-2352
-
-
Sunyach, C.1
Michaud, M.2
Arnoux, T.3
-
55
-
-
84893824003
-
A phase II-III trial of olesoxime in subjects with amyotrophic lateral sclerosis
-
Lenglet T, Lacomblez L, Abitbol JL, et al. A phase II-III trial of olesoxime in subjects with amyotrophic lateral sclerosis. Eur J Neurol. 2014;21(3):529-536.
-
(2014)
Eur J Neurol
, vol.21
, Issue.3
, pp. 529-536
-
-
Lenglet, T.1
Lacomblez, L.2
Abitbol, J.L.3
-
56
-
-
84911007903
-
Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: Restorative effects of olesoxime
-
Eckmann J, Clemens LE, Eckert SH, et al. Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: restorative effects of olesoxime. Mol Neurobiol. 2014;50(1): 107-118.
-
(2014)
Mol Neurobiol
, vol.50
, Issue.1
, pp. 107-118
-
-
Eckmann, J.1
Clemens, L.E.2
Eckert, S.H.3
-
57
-
-
84936751125
-
Olesoxime treatment inhibits the formation of mHtt fragments through suppression of calpain activity, and leads to behavioral and neurological improvements in the BACHD rat
-
Clemens L, Weber J, Wlodkowski TT, et al. Olesoxime treatment inhibits the formation of mHtt fragments through suppression of calpain activity, and leads to behavioral and neurological improvements in the BACHD rat. J Neurol Neurosurg Psych. 2014;85(Suppl 1):A62.
-
(2014)
J Neurol Neurosurg Psych
, vol.85
-
-
Clemens, L.1
Weber, J.2
Wlodkowski, T.T.3
-
58
-
-
77953283266
-
Metallothioneins and copper metabolism are candidate therapeutic targets in Huntington's disease
-
Hands SL, Mason R, Sajjad MU, Giorgini F, Wyttenbach A. Metallothioneins and copper metabolism are candidate therapeutic targets in Huntington's disease. Biochem Soc Trans. 2010;38(2):552-558.
-
(2010)
Biochem Soc Trans
, vol.38
, Issue.2
, pp. 552-558
-
-
Hands, S.L.1
Mason, R.2
Sajjad, M.U.3
Giorgini, F.4
Wyttenbach, A.5
-
59
-
-
84885372632
-
PBT2 reduces toxicity in a C. elegans model of polyQ aggregation and extends lifespan, reduces striatal atrophy and improves motor performance in the R6/2 mouse model of Huntington's disease
-
Chernya R, Aytona S, Finkelsteina D, Busha A, McColla G, Massac S. PBT2 reduces toxicity in a C. elegans model of polyQ aggregation and extends lifespan, reduces striatal atrophy and improves motor performance in the R6/2 mouse model of Huntington's disease. J Huntingtons Dis. 2012;1(2):211-219.
-
(2012)
J Huntingtons Dis
, vol.1
, Issue.2
, pp. 211-219
-
-
Chernya, R.1
Aytona, S.2
Finkelsteina, D.3
Busha, A.4
McColla, G.5
Massac, S.6
-
60
-
-
48949098573
-
Safety, efficacy, and biomarker findings of PBT2 in targeting Aβ as a modifying therapy for Alzheimer's disease: A phase IIA, double-blind, randomised, placebo-controlled trial
-
Lannfelt L, Blennow K, Zetterberg H, et al. Safety, efficacy, and biomarker findings of PBT2 in targeting Aβ as a modifying therapy for Alzheimer's disease: a phase IIA, double-blind, randomised, placebo-controlled trial. Lancet Neurol. 2008;7(9):779-786.
-
(2008)
Lancet Neurol
, vol.7
, Issue.9
, pp. 779-786
-
-
Lannfelt, L.1
Blennow, K.2
Zetterberg, H.3
-
61
-
-
84907923806
-
Clinical trials in Huntington's disease: Interventions in early clinical development and newer methodological approaches
-
Sampaio C, Borowsky B, Reilmann R. Clinical trials in Huntington's disease: interventions in early clinical development and newer methodological approaches. Mov Disord. 2014;29(11):1419-1428.
-
(2014)
Mov Disord
, vol.29
, Issue.11
, pp. 1419-1428
-
-
Sampaio, C.1
Borowsky, B.2
Reilmann, R.3
-
62
-
-
84871925048
-
Kynurenines in the CNS: Recent advances and new questions
-
Vécsei L, Szalárdy L, Fülöp F, Toldi J. Kynurenines in the CNS: recent advances and new questions. Nat Rev Drug Discov. 2013;12(1): 64-82.
-
(2013)
Nat Rev Drug Discov
, vol.12
, Issue.1
, pp. 64-82
-
-
Vécsei, L.1
Szalárdy, L.2
Fülöp, F.3
Toldi, J.4
-
63
-
-
79958021894
-
Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration
-
Zwilling D, Huang SY, Sathyasaikumar KV, et al. Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration. Cell. 2011;145(6):863-874.
-
(2011)
Cell
, vol.145
, Issue.6
, pp. 863-874
-
-
Zwilling, D.1
Huang, S.Y.2
Sathyasaikumar, K.V.3
-
64
-
-
49249089029
-
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease
-
Björkqvist M, Wild EJ, Thiele J, et al. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med. 2008;205(8):1869-1877.
-
(2008)
J Exp Med
, vol.205
, Issue.8
, pp. 1869-1877
-
-
Björkqvist, M.1
Wild, E.J.2
Thiele, J.3
-
65
-
-
84894545327
-
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation
-
Träger U, Andre R, Lahiri N, et al. HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. Brain. 2014;137(Pt 3):819-833.
-
(2014)
Brain
, vol.137
, pp. 819-833
-
-
Träger, U.1
Andre, R.2
Lahiri, N.3
-
66
-
-
84865863065
-
Reduced astrocytic NF-κB activation by laquinimod protects from cuprizone-induced demyelination
-
Brück W, Pförtner R, Pham T, et al. Reduced astrocytic NF-κB activation by laquinimod protects from cuprizone-induced demyelination. Acta Neuropathol. 2012;124(3):411-424.
-
(2012)
Acta Neuropathol
, vol.124
, Issue.3
, pp. 411-424
-
-
Brück, W.1
Pförtner, R.2
Pham, T.3
-
67
-
-
84858217865
-
Placebo-controlled trial of oral laquinimod for multiple sclerosis
-
Comi G, Jeffery D, Kappos L, et al. Placebo-controlled trial of oral laquinimod for multiple sclerosis. N Engl J Med. 2012;366(11):1000-1009.
-
(2012)
N Engl J Med
, vol.366
, Issue.11
, pp. 1000-1009
-
-
Comi, G.1
Jeffery, D.2
Kappos, L.3
-
68
-
-
84906537932
-
Placebo-controlled trial of oral laquinimod in multiple sclerosis: MRI evidence of an effect on brain tissue damage
-
Filippi M, Rocca MA, Pagani E, et al. Placebo-controlled trial of oral laquinimod in multiple sclerosis: MRI evidence of an effect on brain tissue damage. J Neurol Neurosurg Psychiatry. 2014;85(8):851-858.
-
(2014)
J Neurol Neurosurg Psychiatry
, vol.85
, Issue.8
, pp. 851-858
-
-
Filippi, M.1
Rocca, M.A.2
Pagani, E.3
-
69
-
-
33846809051
-
A systematic review of the treatment studies in Huntington's disease since 1990
-
Bonelli RM, Hofmann P. A systematic review of the treatment studies in Huntington's disease since 1990. Expert Opin Pharmacother. 2007; 8(2):141-153.
-
(2007)
Expert Opin Pharmacother
, vol.8
, Issue.2
, pp. 141-153
-
-
Bonelli, R.M.1
Hofmann, P.2
-
70
-
-
84863641230
-
An International survey-based algorithm for the pharmacologic treatment of chorea in Huntington's disease
-
RRN1260
-
Burgunder JM, Guttman M, Perlman S, Goodman N, van Kammen DP, Goodman L. An International survey-based algorithm for the pharmacologic treatment of chorea in Huntington's disease. PLoS Curr. 2011;3:RRN1260.
-
(2011)
PLoS Curr
, vol.3
-
-
Burgunder, J.M.1
Guttman, M.2
Perlman, S.3
Goodman, N.4
van Kammen, D.P.5
Goodman, L.6
-
71
-
-
33645798913
-
Tetrabenazine as antichorea therapy in Huntington disease: A randomized controlled trial
-
Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology. 2006; 66(3):366-372.
-
(2006)
Neurology
, vol.66
, Issue.3
, pp. 366-372
-
-
-
72
-
-
41249090997
-
A study of chorea after tetrabenazine withdrawal in patients with Huntington disease
-
Frank S, Ondo W, Fahn S, et al. A study of chorea after tetrabenazine withdrawal in patients with Huntington disease. Clin Neuropharmacol. 2008;31(3):127-133.
-
(2008)
Clin Neuropharmacol
, vol.31
, Issue.3
, pp. 127-133
-
-
Frank, S.1
Ondo, W.2
Fahn, S.3
-
73
-
-
84872512504
-
A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease
-
HORIZON Investigators of the Huntington Study Group and European Huntington's Disease Network. A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease. Arch Neurol. JAMA Neurol. 2013;70(1):25-33.
-
(2013)
Arch Neurol. JAMA Neurol
, vol.70
, Issue.1
, pp. 25-33
-
-
-
74
-
-
81255195330
-
Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): A phase 3, randomised, double-blind, placebo-controlled trial
-
de Yebenes JG, Landwehrmeyer B, Squitieri F, et al. Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2011;10(12):1049-1057.
-
(2011)
Lancet Neurol
, vol.10
, Issue.12
, pp. 1049-1057
-
-
de Yebenes, J.G.1
Landwehrmeyer, B.2
Squitieri, F.3
-
75
-
-
84884724757
-
A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease
-
Huntington Study Group HART Investigators. A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease. Mov Disord. 2013;28(10):1407-1415.
-
(2013)
Mov Disord
, vol.28
, Issue.10
, pp. 1407-1415
-
-
-
76
-
-
34748821960
-
Myopathy as a first symptom of Huntington's disease in a marathon runner
-
Kosinski CM, Schlangen C, Gellerich FN, et al. Myopathy as a first symptom of Huntington's disease in a marathon runner. Mov Disord. 2007;22(11):1637-1640.
-
(2007)
Mov Disord
, vol.22
, Issue.11
, pp. 1637-1640
-
-
Kosinski, C.M.1
Schlangen, C.2
Gellerich, F.N.3
-
78
-
-
84936787937
-
Myostatin inhibition as a novel approach to targeting muscle pathology in HD
-
Mielcarek M, Rattray I, Osborne G, et al. Myostatin inhibition as a novel approach to targeting muscle pathology in HD. J Neurol Neurosurg Psych. 2014;85(Suppl 1):A97.
-
(2014)
J Neurol Neurosurg Psych
, vol.85
-
-
Mielcarek, M.1
Rattray, I.2
Osborne, G.3
|