-
1
-
-
0021921176
-
Senile plaque amyloid, paired helical filaments, and cerebrovascular amyloid in Alzheimer's disease are all deposits of the same protein
-
M. Kidd et al. Senile plaque amyloid, paired helical filaments, and cerebrovascular amyloid in Alzheimer's disease are all deposits of the same protein Lancet 1 1985 278
-
(1985)
Lancet
, vol.1
, pp. 278
-
-
Kidd, M.1
-
2
-
-
0030882856
-
Alpha-synuclein in Lewy bodies
-
M.G. Spillantini et al. Alpha-synuclein in Lewy bodies Nature 388 1997 839 840
-
(1997)
Nature
, vol.388
, pp. 839-840
-
-
Spillantini, M.G.1
-
3
-
-
41149180753
-
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
-
J. Sreedharan et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis Science 319 2008 1668 1672
-
(2008)
Science
, vol.319
, pp. 1668-1672
-
-
Sreedharan, J.1
-
4
-
-
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
-
The Huntington's Disease Collaborative Research Group A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes Cell 72 1993 971 983
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
5
-
-
33846225133
-
Huntington's disease
-
F.O. Walker Huntington's disease Lancet 369 2007 218 228
-
(2007)
Lancet
, vol.369
, pp. 218-228
-
-
Walker, F.O.1
-
6
-
-
84863833900
-
Network organization of the huntingtin proteomic interactome in mammalian brain
-
D.I. Shirasaki et al. Network organization of the huntingtin proteomic interactome in mammalian brain Neuron 75 2012 41 57
-
(2012)
Neuron
, vol.75
, pp. 41-57
-
-
Shirasaki, D.I.1
-
7
-
-
34249715853
-
Huntingtin interacting proteins are genetic modifiers of neurodegeneration
-
L.S. Kaltenbach et al. Huntingtin interacting proteins are genetic modifiers of neurodegeneration PLoS Genet. 3 2007 e82
-
(2007)
PLoS Genet.
, vol.3
, pp. 82
-
-
Kaltenbach, L.S.1
-
8
-
-
0041656292
-
The hunt for huntingtin function: Interaction partners tell many different stories
-
P. Harjes, and E.E. Wanker The hunt for huntingtin function: interaction partners tell many different stories Trends Biochem. Sci. 28 2003 425 433
-
(2003)
Trends Biochem. Sci.
, vol.28
, pp. 425-433
-
-
Harjes, P.1
Wanker, E.E.2
-
9
-
-
28644433087
-
Normal huntingtin function: An alternative approach to Huntington's disease
-
E. Cattaneo et al. Normal huntingtin function: an alternative approach to Huntington's disease Nat. Rev. Neurosci. 6 2005 919 930
-
(2005)
Nat. Rev. Neurosci.
, vol.6
, pp. 919-930
-
-
Cattaneo, E.1
-
10
-
-
84855499458
-
Huntington's disease: Molecular basis of neurodegeneration
-
D.C. Rubinsztein, and J. Carmichael Huntington's disease: molecular basis of neurodegeneration Expert Rev. Mol. Med. 5 2003 1 21
-
(2003)
Expert Rev. Mol. Med.
, vol.5
, pp. 1-21
-
-
Rubinsztein, D.C.1
Carmichael, J.2
-
11
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
M. Arrasate et al. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death Nature 431 2004 805 810
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
-
12
-
-
84883204078
-
Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration
-
A.S. Tsvetkov et al. Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration Nat. Chem. Biol. 9 2013 586 592
-
(2013)
Nat. Chem. Biol.
, vol.9
, pp. 586-592
-
-
Tsvetkov, A.S.1
-
13
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
A. Yamamoto et al. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease Cell 101 2000 57 66
-
(2000)
Cell
, vol.101
, pp. 57-66
-
-
Yamamoto, A.1
-
14
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
-
S. Zeitlin et al. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue Nat. Genet. 11 1995 155 163
-
(1995)
Nat. Genet.
, vol.11
, pp. 155-163
-
-
Zeitlin, S.1
-
15
-
-
0029082383
-
Inactivation of the mouse Huntington's disease gene homolog Hdh
-
M.P. Duyao et al. Inactivation of the mouse Huntington's disease gene homolog Hdh Science 269 1995 407 410
-
(1995)
Science
, vol.269
, pp. 407-410
-
-
Duyao, M.P.1
-
16
-
-
67349100160
-
Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice
-
R.L. Boudreau et al. Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice Mol. Ther. 17 2009 1053 1063
-
(2009)
Mol. Ther.
, vol.17
, pp. 1053-1063
-
-
Boudreau, R.L.1
-
17
-
-
84860192454
-
Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum
-
R. Grondin et al. Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum Brain 135 2012 1197 1209
-
(2012)
Brain
, vol.135
, pp. 1197-1209
-
-
Grondin, R.1
-
18
-
-
84877139837
-
A novel human embryonic stem cell-derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT-dependent neurodegeneration
-
B. Lu, and J. Palacino A novel human embryonic stem cell-derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT-dependent neurodegeneration FASEB J. 27 2013 1820 1829
-
(2013)
FASEB J.
, vol.27
, pp. 1820-1829
-
-
Lu, B.1
Palacino, J.2
-
19
-
-
20244378556
-
RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
-
S.Q. Harper et al. RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model Proc. Natl. Acad. Sci. U.S.A. 102 2005 5820 5825
-
(2005)
Proc. Natl. Acad. Sci. U.S.A.
, vol.102
, pp. 5820-5825
-
-
Harper, S.Q.1
-
20
-
-
25144464388
-
Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice
-
E. Rodriguez-Lebron et al. Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice Mol. Ther. 12 2005 618 633
-
(2005)
Mol. Ther.
, vol.12
, pp. 618-633
-
-
Rodriguez-Lebron, E.1
-
21
-
-
36749033738
-
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
-
M. DiFiglia et al. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits Proc. Natl. Acad. Sci. U.S.A. 104 2007 17204 17209
-
(2007)
Proc. Natl. Acad. Sci. U.S.A.
, vol.104
, pp. 17204-17209
-
-
Difiglia, M.1
-
22
-
-
47549105506
-
Identification and allele-specific silencing of the mutant huntingtin allele in Huntington's disease patient-derived fibroblasts
-
P.H. van Bilsen et al. Identification and allele-specific silencing of the mutant huntingtin allele in Huntington's disease patient-derived fibroblasts Hum. Gene Ther. 19 2008 710 719
-
(2008)
Hum. Gene Ther.
, vol.19
, pp. 710-719
-
-
Van Bilsen, P.H.1
-
23
-
-
67349159137
-
Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients
-
E.L. Pfister et al. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients Curr. Biol. 19 2009 774 778
-
(2009)
Curr. Biol.
, vol.19
, pp. 774-778
-
-
Pfister, E.L.1
-
24
-
-
57249086448
-
Allele-specific silencing of mutant Huntington's disease gene
-
Y. Zhang et al. Allele-specific silencing of mutant Huntington's disease gene J. Neurochem. 108 2009 82 90
-
(2009)
J. Neurochem.
, vol.108
, pp. 82-90
-
-
Zhang, Y.1
-
25
-
-
78649359013
-
Allele-selective inhibition of huntingtin expression by switching to an miRNA-like RNAi mechanism
-
J. Hu et al. Allele-selective inhibition of huntingtin expression by switching to an miRNA-like RNAi mechanism Chem. Biol. 17 2010 1183 1188
-
(2010)
Chem. Biol.
, vol.17
, pp. 1183-1188
-
-
Hu, J.1
-
26
-
-
78649379362
-
Allele-selective inhibition of mutant huntingtin expression with antisense oligonucleotides targeting the expanded CAG repeat
-
K.T. Gagnon et al. Allele-selective inhibition of mutant huntingtin expression with antisense oligonucleotides targeting the expanded CAG repeat Biochemistry 49 2010 10166 10178
-
(2010)
Biochemistry
, vol.49
, pp. 10166-10178
-
-
Gagnon, K.T.1
-
27
-
-
82955237522
-
Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene/allele-specific silencing of mutant huntingtin
-
J.B. Carroll et al. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene/allele-specific silencing of mutant huntingtin Mol. Ther. 19 2011 2178 2185
-
(2011)
Mol. Ther.
, vol.19
, pp. 2178-2185
-
-
Carroll, J.B.1
-
28
-
-
84862663712
-
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis
-
H.B. Kordasiewicz et al. Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis Neuron 74 2012 1031 1044
-
(2012)
Neuron
, vol.74
, pp. 1031-1044
-
-
Kordasiewicz, H.B.1
-
29
-
-
84865688581
-
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression
-
D. Yu et al. Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression Cell 150 2012 895 908
-
(2012)
Cell
, vol.150
, pp. 895-908
-
-
Yu, D.1
-
30
-
-
65249131740
-
Sustained effects of nonallele-specific Huntingtin silencing
-
V. Drouet et al. Sustained effects of nonallele-specific Huntingtin silencing Ann. Neurol. 65 2009 276 285
-
(2009)
Ann. Neurol.
, vol.65
, pp. 276-285
-
-
Drouet, V.1
-
31
-
-
82955199935
-
Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington's disease
-
J.L. McBride et al. Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington's disease Mol. Ther. 19 2011 2152 2162
-
(2011)
Mol. Ther.
, vol.19
, pp. 2152-2162
-
-
McBride, J.L.1
-
32
-
-
27644596641
-
What is the role of protein aggregation in neurodegeneration?
-
C.A. Ross, and M.A. Poirier What is the role of protein aggregation in neurodegeneration? Nat. Rev. Mol. Cell Biol. 6 2005 891 898
-
(2005)
Nat. Rev. Mol. Cell Biol.
, vol.6
, pp. 891-898
-
-
Ross, C.A.1
Poirier, M.A.2
-
33
-
-
0033119123
-
Nuclear and neuropil aggregates in Huntington's disease: Relationship to neuropathology
-
C.A. Gutekunst et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology J. Neurosci. 19 1999 2522 2534
-
(1999)
J. Neurosci.
, vol.19
, pp. 2522-2534
-
-
Gutekunst, C.A.1
-
34
-
-
0033614770
-
Are there multiple pathways in the pathogenesis of Huntington's disease?
-
N. Aronin et al. Are there multiple pathways in the pathogenesis of Huntington's disease? Phillos. Trans. R. Soc. Lond. B: Biol. Sci. 354 1999 995 1003
-
(1999)
Phillos. Trans. R. Soc. Lond. B: Biol. Sci.
, vol.354
, pp. 995-1003
-
-
Aronin, N.1
-
35
-
-
84887792874
-
Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress
-
J. Leitman et al. Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress Nat. Commun. 4 2013 2753
-
(2013)
Nat. Commun.
, vol.4
, pp. 2753
-
-
Leitman, J.1
-
36
-
-
77950682093
-
Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease
-
X. Li et al. Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease J. Neurosci. 30 2010 4552 4561
-
(2010)
J. Neurosci.
, vol.30
, pp. 4552-4561
-
-
Li, X.1
-
37
-
-
84859760185
-
Native mutant huntingtin in human brain: Evidence for prevalence of full-length monomer
-
E. Sapp et al. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer J. Biol. Chem. 287 2012 13487 13499
-
(2012)
J. Biol. Chem.
, vol.287
, pp. 13487-13499
-
-
Sapp, E.1
-
38
-
-
81355160169
-
Identifying polyglutamine protein species in situ that best predict neurodegeneration
-
J. Miller et al. Identifying polyglutamine protein species in situ that best predict neurodegeneration Nat. Chem. Biol. 7 2011 925 934
-
(2011)
Nat. Chem. Biol.
, vol.7
, pp. 925-934
-
-
Miller, J.1
-
39
-
-
0033280667
-
Vacuolar import of proteins and organelles from the cytoplasm
-
D.J. Klionsky, and Y. Ohsumi Vacuolar import of proteins and organelles from the cytoplasm Annu. Rev. Cell Dev. Biol. 15 1999 1 32
-
(1999)
Annu. Rev. Cell Dev. Biol.
, vol.15
, pp. 1-32
-
-
Klionsky, D.J.1
Ohsumi, Y.2
-
40
-
-
33847705665
-
Role of ubiquitin- and Ubl-binding proteins in cell signaling
-
V. Kirkin, and I. Dikic Role of ubiquitin- and Ubl-binding proteins in cell signaling Curr. Opin. Cell Biol. 19 2007 199 205
-
(2007)
Curr. Opin. Cell Biol.
, vol.19
, pp. 199-205
-
-
Kirkin, V.1
Dikic, I.2
-
41
-
-
37649005234
-
Autophagy in the pathogenesis of disease
-
B. Levine, and G. Kroemer Autophagy in the pathogenesis of disease Cell 132 2008 27 42
-
(2008)
Cell
, vol.132
, pp. 27-42
-
-
Levine, B.1
Kroemer, G.2
-
42
-
-
36849089101
-
Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice
-
M. Komatsu et al. Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice Cell 131 2007 1149 1163
-
(2007)
Cell
, vol.131
, pp. 1149-1163
-
-
Komatsu, M.1
-
43
-
-
27944504351
-
P62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death
-
G. Bjorkoy et al. p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death J. Cell Biol. 171 2005 603 614
-
(2005)
J. Cell Biol.
, vol.171
, pp. 603-614
-
-
Bjorkoy, G.1
-
44
-
-
77950903972
-
The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein Alfy
-
M. Filimonenko et al. The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein Alfy Mol. Cell 38 2010 265 279
-
(2010)
Mol. Cell
, vol.38
, pp. 265-279
-
-
Filimonenko, M.1
-
45
-
-
80955177196
-
TFEB links autophagy to lysosomal biogenesis
-
C. Settembre et al. TFEB links autophagy to lysosomal biogenesis Science 332 2011 1429 1433
-
(2011)
Science
, vol.332
, pp. 1429-1433
-
-
Settembre, C.1
-
46
-
-
84863923855
-
PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function
-
T. Tsunemi et al. PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function Sci. Transl. Med. 4 2012 142ra197
-
(2012)
Sci. Transl. Med.
, vol.4
-
-
Tsunemi, T.1
-
47
-
-
15744387323
-
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes
-
N.R. Jana et al. Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes J. Biol. Chem. 280 2005 11635 11640
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 11635-11640
-
-
Jana, N.R.1
-
48
-
-
77749319356
-
Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein
-
P.O. Bauer et al. Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein Nat. Biotechnol. 28 2010 256 263
-
(2010)
Nat. Biotechnol.
, vol.28
, pp. 256-263
-
-
Bauer, P.O.1
-
49
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
B. Ravikumar et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease Nat. Genet. 36 2004 585 595
-
(2004)
Nat. Genet.
, vol.36
, pp. 585-595
-
-
Ravikumar, B.1
-
50
-
-
57649227693
-
Rapamycin and mTOR-independent autophagy inducers ameliorate toxicity of polyglutamine-expanded huntingtin and related proteinopathies
-
S. Sarkar et al. Rapamycin and mTOR-independent autophagy inducers ameliorate toxicity of polyglutamine-expanded huntingtin and related proteinopathies Cell Death Differ. 16 2009 46 56
-
(2009)
Cell Death Differ.
, vol.16
, pp. 46-56
-
-
Sarkar, S.1
-
51
-
-
0038364056
-
Raised intracellular glucose concentrations reduce aggregation and cell death caused by mutant huntingtin exon 1 by decreasing mTOR phosphorylation and inducing autophagy
-
B. Ravikumar et al. Raised intracellular glucose concentrations reduce aggregation and cell death caused by mutant huntingtin exon 1 by decreasing mTOR phosphorylation and inducing autophagy Hum. Mol. Genet. 12 2003 985 994
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 985-994
-
-
Ravikumar, B.1
-
52
-
-
53749104349
-
Akt inhibition promotes autophagy and sensitizes PTEN-null tumors to lysosomotropic agents
-
M. Degtyarev et al. Akt inhibition promotes autophagy and sensitizes PTEN-null tumors to lysosomotropic agents J. Cell Biol. 183 2008 101 116
-
(2008)
J. Cell Biol.
, vol.183
, pp. 101-116
-
-
Degtyarev, M.1
-
53
-
-
65949086045
-
Atg5 regulates phenethyl isothiocyanate-induced autophagic and apoptotic cell death in human prostate cancer cells
-
A. Bommareddy et al. Atg5 regulates phenethyl isothiocyanate-induced autophagic and apoptotic cell death in human prostate cancer cells Cancer Res. 69 2009 3704 3712
-
(2009)
Cancer Res.
, vol.69
, pp. 3704-3712
-
-
Bommareddy, A.1
-
54
-
-
57349180534
-
Antioxidants in central nervous system diseases: Preclinical promise and translational challenges
-
C.D. Kamat et al. Antioxidants in central nervous system diseases: preclinical promise and translational challenges J. Alzheimers Dis. 15 2008 473 493
-
(2008)
J. Alzheimers Dis.
, vol.15
, pp. 473-493
-
-
Kamat, C.D.1
-
55
-
-
77955383021
-
Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease
-
B.R. Underwood et al. Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease Hum. Mol. Genet. 19 2010 3413 3429
-
(2010)
Hum. Mol. Genet.
, vol.19
, pp. 3413-3429
-
-
Underwood, B.R.1
-
56
-
-
25444483066
-
Lithium induces autophagy by inhibiting inositol monophosphatase
-
S. Sarkar et al. Lithium induces autophagy by inhibiting inositol monophosphatase J. Cell Biol. 170 2005 1101 1111
-
(2005)
J. Cell Biol.
, vol.170
, pp. 1101-1111
-
-
Sarkar, S.1
-
57
-
-
37849042536
-
A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin
-
S. Sarkar et al. A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin Hum. Mol. Genet. 17 2008 170 178
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 170-178
-
-
Sarkar, S.1
-
58
-
-
42249106042
-
Novel targets for Huntington's disease in an mTOR-independent autophagy pathway
-
A. Williams et al. Novel targets for Huntington's disease in an mTOR-independent autophagy pathway Nat. Chem. Biol. 4 2008 295 305
-
(2008)
Nat. Chem. Biol.
, vol.4
, pp. 295-305
-
-
Williams, A.1
-
59
-
-
77953486943
-
Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease
-
C. Rose et al. Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease Hum. Mol. Genet. 19 2010 2144 2153
-
(2010)
Hum. Mol. Genet.
, vol.19
, pp. 2144-2153
-
-
Rose, C.1
-
60
-
-
0027495705
-
Dependence of hepatocytic autophagy on intracellularly sequestered calcium
-
P.B. Gordon et al. Dependence of hepatocytic autophagy on intracellularly sequestered calcium J. Biol. Chem. 268 1993 26107 26112
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 26107-26112
-
-
Gordon, P.B.1
-
61
-
-
34247161367
-
Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and α-synuclein
-
S. Sarkar et al. Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and α-synuclein J. Biol. Chem. 282 2007 5641 5652
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 5641-5652
-
-
Sarkar, S.1
-
62
-
-
37649024076
-
Small molecule regulators of autophagy identified by an image-based high-throughput screen
-
L. Zhang et al. Small molecule regulators of autophagy identified by an image-based high-throughput screen Proc. Natl. Acad. Sci. U.S.A. 104 2007 19023 19028
-
(2007)
Proc. Natl. Acad. Sci. U.S.A.
, vol.104
, pp. 19023-19028
-
-
Zhang, L.1
-
63
-
-
84860681788
-
Autophagy and polyglutamine diseases
-
M. Jimenez-Sanchez et al. Autophagy and polyglutamine diseases Prog. Neurobiol. 97 2012 67 82
-
(2012)
Prog. Neurobiol.
, vol.97
, pp. 67-82
-
-
Jimenez-Sanchez, M.1
-
64
-
-
56749170677
-
Autophagic cell death: The story of a misnomer
-
G. Kroemer, and B. Levine Autophagic cell death: the story of a misnomer Nat. Rev. Mol. Cell Biol. 9 2008 1004 1010
-
(2008)
Nat. Rev. Mol. Cell Biol.
, vol.9
, pp. 1004-1010
-
-
Kroemer, G.1
Levine, B.2
-
65
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
N.F. Bence et al. Impairment of the ubiquitin-proteasome system by protein aggregation Science 292 2001 1552 1555
-
(2001)
Science
, vol.292
, pp. 1552-1555
-
-
Bence, N.F.1
-
66
-
-
1842766144
-
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
-
P. Venkatraman et al. Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins Mol. Cell 14 2004 95 104
-
(2004)
Mol. Cell
, vol.14
, pp. 95-104
-
-
Venkatraman, P.1
-
67
-
-
67650258745
-
Enhanced degradation of mutant huntingtin by rho kinase inhibition is mediated through activation of proteasome and macroautophagy
-
P.O. Bauer, and N. Nukina Enhanced degradation of mutant huntingtin by rho kinase inhibition is mediated through activation of proteasome and macroautophagy Autophagy 5 2009 747 748
-
(2009)
Autophagy
, vol.5
, pp. 747-748
-
-
Bauer, P.O.1
Nukina, N.2
-
68
-
-
72149124383
-
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
-
L.M. Thompson et al. IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome J. Cell Biol. 187 2009 1083 1099
-
(2009)
J. Cell Biol.
, vol.187
, pp. 1083-1099
-
-
Thompson, L.M.1
-
69
-
-
84884567375
-
Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes
-
K. Juenemann et al. Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes J. Biol. Chem. 288 2013 27068 27084
-
(2013)
J. Biol. Chem.
, vol.288
, pp. 27068-27084
-
-
Juenemann, K.1
-
70
-
-
84876917897
-
Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance
-
B. Lu et al. Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance Nat. Neurosci. 16 2013 562 570
-
(2013)
Nat. Neurosci.
, vol.16
, pp. 562-570
-
-
Lu, B.1
-
71
-
-
0035824559
-
Targeting of NEDD8 and its conjugates for proteasomal degradation by NUB1
-
T. Kamitani et al. Targeting of NEDD8 and its conjugates for proteasomal degradation by NUB1 J. Biol. Chem. 276 2001 46655 46660
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 46655-46660
-
-
Kamitani, T.1
-
72
-
-
0035827621
-
NUB1, a NEDD8-interacting protein, is induced by interferon and down-regulates the NEDD8 expression
-
K. Kito et al. NUB1, a NEDD8-interacting protein, is induced by interferon and down-regulates the NEDD8 expression J. Biol. Chem. 276 2001 20603 20609
-
(2001)
J. Biol. Chem.
, vol.276
, pp. 20603-20609
-
-
Kito, K.1
-
73
-
-
0041856380
-
Regulation of the NEDD8 conjugation system by a splicing variant, NUB1L
-
T. Tanaka et al. Regulation of the NEDD8 conjugation system by a splicing variant, NUB1L J. Biol. Chem. 278 2003 32905 32913
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 32905-32913
-
-
Tanaka, T.1
-
74
-
-
1942533448
-
NEDD8 ultimate buster-1L interacts with the ubiquitin-like protein FAT10 and accelerates its degradation
-
M.S. Hipp et al. NEDD8 ultimate buster-1L interacts with the ubiquitin-like protein FAT10 and accelerates its degradation J. Biol. Chem. 279 2004 16503 16510
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 16503-16510
-
-
Hipp, M.S.1
-
75
-
-
84876934388
-
NUB1 snubs huntingtin toxicity
-
R. Aron et al. NUB1 snubs huntingtin toxicity Nat. Neurosci. 16 2013 523 525
-
(2013)
Nat. Neurosci.
, vol.16
, pp. 523-525
-
-
Aron, R.1
-
76
-
-
2442529664
-
Cullin-based ubiquitin ligases: Cul3-BTB complexes join the family
-
L. Pintard et al. Cullin-based ubiquitin ligases: Cul3-BTB complexes join the family EMBO J. 23 2004 1681 1687
-
(2004)
EMBO J.
, vol.23
, pp. 1681-1687
-
-
Pintard, L.1
-
77
-
-
74049138620
-
The Cul3-KLHL21 E3 ubiquitin ligase targets aurora B to midzone microtubules in anaphase and is required for cytokinesis
-
S. Maerki et al. The Cul3-KLHL21 E3 ubiquitin ligase targets aurora B to midzone microtubules in anaphase and is required for cytokinesis J. Cell Biol. 187 2009 791 800
-
(2009)
J. Cell Biol.
, vol.187
, pp. 791-800
-
-
Maerki, S.1
-
78
-
-
34249305132
-
A Cul3-based E3 ligase removes Aurora B from mitotic chromosomes, regulating mitotic progression and completion of cytokinesis in human cells
-
I. Sumara et al. A Cul3-based E3 ligase removes Aurora B from mitotic chromosomes, regulating mitotic progression and completion of cytokinesis in human cells Dev. Cell 12 2007 887 900
-
(2007)
Dev. Cell
, vol.12
, pp. 887-900
-
-
Sumara, I.1
-
79
-
-
0027521002
-
Interferon β-1b is effective in relapsing-remitting multiple sclerosis. II. MRI analysis results of a multicenter, randomized, double-blind, placebo-controlled trial. UBC MS/MRI Study Group and the IFNB Multiple Sclerosis Study Group
-
D.W. Paty, and D.K. Li Interferon β-1b is effective in relapsing-remitting multiple sclerosis. II. MRI analysis results of a multicenter, randomized, double-blind, placebo-controlled trial. UBC MS/MRI Study Group and the IFNB Multiple Sclerosis Study Group Neurology 43 1993 662 667
-
(1993)
Neurology
, vol.43
, pp. 662-667
-
-
Paty, D.W.1
Li, D.K.2
-
80
-
-
84878820326
-
Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice
-
A. Chort et al. Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice Brain 136 2013 1732 1745
-
(2013)
Brain
, vol.136
, pp. 1732-1745
-
-
Chort, A.1
-
81
-
-
77951665859
-
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
-
M. Martinez-Vicente et al. Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease Nat. Neurosci. 13 2010 567 576
-
(2010)
Nat. Neurosci.
, vol.13
, pp. 567-576
-
-
Martinez-Vicente, M.1
-
82
-
-
84859983420
-
Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease
-
M.S. Hipp et al. Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease J. Cell Biol. 196 2012 573 587
-
(2012)
J. Cell Biol.
, vol.196
, pp. 573-587
-
-
Hipp, M.S.1
-
83
-
-
72149107077
-
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice
-
X. Gu et al. Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice Neuron 64 2009 828 840
-
(2009)
Neuron
, vol.64
, pp. 828-840
-
-
Gu, X.1
-
84
-
-
63049132756
-
Acetylation targets mutant huntingtin to autophagosomes for degradation
-
H. Jeong et al. Acetylation targets mutant huntingtin to autophagosomes for degradation Cell 137 2009 60 72
-
(2009)
Cell
, vol.137
, pp. 60-72
-
-
Jeong, H.1
-
85
-
-
78349301194
-
Antibody therapy in neurodegenerative disease
-
A.L. Southwell, and P.H. Patterson Antibody therapy in neurodegenerative disease Rev. Neurosci. 21 2010 273 287
-
(2010)
Rev. Neurosci.
, vol.21
, pp. 273-287
-
-
Southwell, A.L.1
Patterson, P.H.2
-
86
-
-
84855478434
-
A screen for enhancers of clearance identifies huntingtin as a heat shock protein 90 (Hsp90) client protein
-
B. Baldo et al. A screen for enhancers of clearance identifies huntingtin as a heat shock protein 90 (Hsp90) client protein J. Biol. Chem. 287 2012 1406 1414
-
(2012)
J. Biol. Chem.
, vol.287
, pp. 1406-1414
-
-
Baldo, B.1
-
87
-
-
77955870526
-
A genome-wide RNA interference screen for modifiers of aggregates formation by mutant Huntingtin in Drosophila
-
S. Zhang et al. A genome-wide RNA interference screen for modifiers of aggregates formation by mutant Huntingtin in Drosophila Genetics 184 2010 1165 1179
-
(2010)
Genetics
, vol.184
, pp. 1165-1179
-
-
Zhang, S.1
-
88
-
-
77955500335
-
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease
-
J.P. Miller et al. Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease Neuron 67 2010 199 212
-
(2010)
Neuron
, vol.67
, pp. 199-212
-
-
Miller, J.P.1
-
89
-
-
70349675573
-
RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation
-
J. Doumanis et al. RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation PLoS ONE 4 2009 e7275
-
(2009)
PLoS ONE
, vol.4
, pp. 7275
-
-
Doumanis, J.1
-
90
-
-
84864628471
-
Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes
-
H.D. The, and iPSC
-
H.D. The, and iPSC. Consortium Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes Cell Stem Cell 11 2012 264 278
-
(2012)
Cell Stem Cell
, vol.11
, pp. 264-278
-
-
-
91
-
-
79551655290
-
Huntington's disease: Can mice lead the way to treatment?
-
Z.R. Crook, and D. Housman Huntington's disease: can mice lead the way to treatment? Neuron 69 2011 423 435
-
(2011)
Neuron
, vol.69
, pp. 423-435
-
-
Crook, Z.R.1
Housman, D.2
-
93
-
-
45149105779
-
Towards a transgenic model of Huntington's disease in a non-human primate
-
S.H. Yang et al. Towards a transgenic model of Huntington's disease in a non-human primate Nature 453 2008 921 924
-
(2008)
Nature
, vol.453
, pp. 921-924
-
-
Yang, S.H.1
-
94
-
-
77957732707
-
Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs
-
D. Yang et al. Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs Hum. Mol. Genet. 19 2010 3983 3994
-
(2010)
Hum. Mol. Genet.
, vol.19
, pp. 3983-3994
-
-
Yang, D.1
-
95
-
-
84868093925
-
Mouse models of polyglutamine diseases in therapeutic approaches: Review and data table. Part II
-
P.M. Switonski et al. Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II Mol. Neurobiol. 46 2012 430 466
-
(2012)
Mol. Neurobiol.
, vol.46
, pp. 430-466
-
-
Switonski, P.M.1
-
97
-
-
0041691176
-
Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats
-
L.B. Menalled et al. Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats J. Comp. Neurol. 465 2003 11 26
-
(2003)
J. Comp. Neurol.
, vol.465
, pp. 11-26
-
-
Menalled, L.B.1
-
98
-
-
38349171781
-
Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases
-
J. Branco et al. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases Hum. Mol. Genet. 17 2008 376 390
-
(2008)
Hum. Mol. Genet.
, vol.17
, pp. 376-390
-
-
Branco, J.1
-
99
-
-
33749633639
-
Modeling polyglutamine pathogenesis in C. Elegans
-
H.R. Brignull et al. Modeling polyglutamine pathogenesis in C. elegans Methods Enzymol. 412 2006 256 282
-
(2006)
Methods Enzymol.
, vol.412
, pp. 256-282
-
-
Brignull, H.R.1
-
100
-
-
84879414055
-
RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1
-
J. Park et al. RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1 Nature 498 2013 325 331
-
(2013)
Nature
, vol.498
, pp. 325-331
-
-
Park, J.1
-
101
-
-
84870686106
-
A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease
-
J.P. Miller et al. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease PLoS Genet. 8 2012 e1003042
-
(2012)
PLoS Genet.
, vol.8
, pp. 1003042
-
-
Miller, J.P.1
-
102
-
-
84887560529
-
Pizotifen activates ERK and provides neuroprotection in vitro and in vivo in models of Huntington's disease
-
M.R. Sarantos et al. Pizotifen activates ERK and provides neuroprotection in vitro and in vivo in models of Huntington's disease J. Huntingtons Dis. 1 2012 195 210
-
(2012)
J. Huntingtons Dis.
, vol.1
, pp. 195-210
-
-
Sarantos, M.R.1
-
103
-
-
84885020652
-
Glutathione peroxidase activity is neuroprotective in models of Huntington's disease
-
R.P. Mason et al. Glutathione peroxidase activity is neuroprotective in models of Huntington's disease Nat. Genet. 45 2013 1249 1254
-
(2013)
Nat. Genet.
, vol.45
, pp. 1249-1254
-
-
Mason, R.P.1
-
104
-
-
70349103737
-
Single-step detection of mutant huntingtin in animal and human tissues: A bioassay for Huntington's disease
-
A. Weiss et al. Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease Anal. Biochem. 395 2009 8 15
-
(2009)
Anal. Biochem.
, vol.395
, pp. 8-15
-
-
Weiss, A.1
-
105
-
-
20044390015
-
A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo
-
X. Zhang et al. A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo Proc. Natl. Acad. Sci. U.S.A. 102 2005 892 897
-
(2005)
Proc. Natl. Acad. Sci. U.S.A.
, vol.102
, pp. 892-897
-
-
Zhang, X.1
-
106
-
-
84859179041
-
A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity
-
M. Banez-Coronel et al. A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity PLoS Genet. 8 2012 e1002481
-
(2012)
PLoS Genet.
, vol.8
, pp. 1002481
-
-
Banez-Coronel, M.1
-
107
-
-
84864631163
-
Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells
-
M.C. An et al. Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells Cell Stem Cell 11 2012 253 263
-
(2012)
Cell Stem Cell
, vol.11
, pp. 253-263
-
-
An, M.C.1
|