-
1
-
-
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, 1993. Cell. 72, 971-83.
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group, 1993. Cell. 72, 971-83.
-
-
-
-
2
-
-
0037947662
-
A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila
-
Apostol B.L., et al. A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc. Natl. Acad. Sci. U. S. A. 100 (2003) 5950-5955
-
(2003)
Proc. Natl. Acad. Sci. U. S. A.
, vol.100
, pp. 5950-5955
-
-
Apostol, B.L.1
-
3
-
-
0042126676
-
Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?
-
Bates G.P., and Hockly E. Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?. Curr. Opin. Neurol. 16 (2003) 465-470
-
(2003)
Curr. Opin. Neurol.
, vol.16
, pp. 465-470
-
-
Bates, G.P.1
Hockly, E.2
-
4
-
-
67649690944
-
Inhibition of rho-kinases enhances the degradation of mutant huntingtin
-
Bauer P.O., et al. Inhibition of rho-kinases enhances the degradation of mutant huntingtin. J. Biol. Chem. 284 (2009) 13153-13164
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 13153-13164
-
-
Bauer, P.O.1
-
5
-
-
43649084649
-
Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics
-
Burnett B.G., et al. Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics. Neurobiol. Dis. 30 (2008) 365-374
-
(2008)
Neurobiol. Dis.
, vol.30
, pp. 365-374
-
-
Burnett, B.G.1
-
6
-
-
53049090989
-
A common motif targets huntingtin and the androgen receptor to the proteasome
-
Chandra S., et al. A common motif targets huntingtin and the androgen receptor to the proteasome. J. Biol. Chem. 283 (2008) 23950-23955
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 23950-23955
-
-
Chandra, S.1
-
7
-
-
0033912716
-
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
-
Chen M., et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat. Med. 6 (2000) 797-801
-
(2000)
Nat. Med.
, vol.6
, pp. 797-801
-
-
Chen, M.1
-
8
-
-
36749022180
-
A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse
-
Chopra V., et al. A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse. Proc. Natl. Acad. Sci. U. S. A. 104 (2007) 16685-16689
-
(2007)
Proc. Natl. Acad. Sci. U. S. A.
, vol.104
, pp. 16685-16689
-
-
Chopra, V.1
-
9
-
-
20244362093
-
CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model
-
Chou S.Y., et al. CGS21680 attenuates symptoms of Huntington's disease in a transgenic mouse model. J. Neurochem. 93 (2005) 310-320
-
(2005)
J. Neurochem.
, vol.93
, pp. 310-320
-
-
Chou, S.Y.1
-
10
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies S.W., et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90 (1997) 537-548
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
-
11
-
-
0036523110
-
Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease
-
Ferrante R.J., et al. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J. Neurosci. 22 (2002) 1592-1599
-
(2002)
J. Neurosci.
, vol.22
, pp. 1592-1599
-
-
Ferrante, R.J.1
-
12
-
-
0142157600
-
Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice
-
Ferrante R.J., et al. Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice. J. Neurosci. 23 (2003) 9418-9427
-
(2003)
J. Neurosci.
, vol.23
, pp. 9418-9427
-
-
Ferrante, R.J.1
-
13
-
-
20844455450
-
Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease
-
Ferrante R.J., et al. Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease. J. Neurosci. 24 (2004) 10335-10342
-
(2004)
J. Neurosci.
, vol.24
, pp. 10335-10342
-
-
Ferrante, R.J.1
-
14
-
-
5444229092
-
Cystamine increases l-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation
-
Fox J.H., et al. Cystamine increases l-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J. Neurochem. 91 (2004) 413-422
-
(2004)
J. Neurochem.
, vol.91
, pp. 413-422
-
-
Fox, J.H.1
-
15
-
-
4644231870
-
A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease
-
Goehler H., et al. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. Mol. Cell. 15 (2004) 853-865
-
(2004)
Mol. Cell.
, vol.15
, pp. 853-865
-
-
Goehler, H.1
-
16
-
-
0036152343
-
Environmental enrichment slows disease progression in R6/2 Huntington's disease mice
-
Hockly E., et al. Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann. Neurol. 51 (2002) 235-242
-
(2002)
Ann. Neurol.
, vol.51
, pp. 235-242
-
-
Hockly, E.1
-
17
-
-
0037452775
-
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
-
Hockly E., et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc. Natl. Acad. Sci. U. S. A. 100 (2003) 2041-2046
-
(2003)
Proc. Natl. Acad. Sci. U. S. A.
, vol.100
, pp. 2041-2046
-
-
Hockly, E.1
-
18
-
-
0141678246
-
Standardization and statistical approaches to therapeutic trials in the R6/2 mouse
-
Hockly E., et al. Standardization and statistical approaches to therapeutic trials in the R6/2 mouse. Brain. Res. Bull. 61 (2003) 469-479
-
(2003)
Brain. Res. Bull.
, vol.61
, pp. 469-479
-
-
Hockly, E.1
-
19
-
-
50249104353
-
Antiepileptic effects of two Rho-kinase inhibitors, Y-27632 and fasudil, in mice
-
Inan S., and Buyukafsar K. Antiepileptic effects of two Rho-kinase inhibitors, Y-27632 and fasudil, in mice. Br. J. Pharmacol. 155 (2008) 44-51
-
(2008)
Br. J. Pharmacol.
, vol.155
, pp. 44-51
-
-
Inan, S.1
Buyukafsar, K.2
-
20
-
-
0034017744
-
Pharmacological properties of Y-27632, a specific inhibitor of rho-associated kinases
-
Ishizaki T., et al. Pharmacological properties of Y-27632, a specific inhibitor of rho-associated kinases. Mol. Pharmacol. 57 (2000) 976-983
-
(2000)
Mol. Pharmacol.
, vol.57
, pp. 976-983
-
-
Ishizaki, T.1
-
21
-
-
18544379477
-
A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila
-
Kazantsev A., et al. A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila. Nat. Genet. 30 (2002) 367-376
-
(2002)
Nat. Genet.
, vol.30
, pp. 367-376
-
-
Kazantsev, A.1
-
22
-
-
34047269594
-
Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy
-
Li M., et al. Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy. J. Biol. Chem. 282 (2007) 3157-3164
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 3157-3164
-
-
Li, M.1
-
23
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini L., et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87 (1996) 493-506
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
-
24
-
-
50249084354
-
Protein misfolding and cardiac disease: establishing cause and effect
-
Pattison J.S., and Robbins J. Protein misfolding and cardiac disease: establishing cause and effect. Autophagy 4 (2008) 821-823
-
(2008)
Autophagy
, vol.4
, pp. 821-823
-
-
Pattison, J.S.1
Robbins, J.2
-
25
-
-
0242657586
-
A rapid cellular FRET assay of polyglutamine aggregation identifies a novel inhibitor
-
Pollitt S.K., et al. A rapid cellular FRET assay of polyglutamine aggregation identifies a novel inhibitor. Neuron 40 (2003) 685-694
-
(2003)
Neuron
, vol.40
, pp. 685-694
-
-
Pollitt, S.K.1
-
26
-
-
33749040671
-
Role of autophagy in the clearance of mutant huntingtin: a step towards therapy?
-
Ravikumar B., and Rubinsztein D.C. Role of autophagy in the clearance of mutant huntingtin: a step towards therapy?. Mol. Aspects Med. 27 (2006) 520-527
-
(2006)
Mol. Aspects Med.
, vol.27
, pp. 520-527
-
-
Ravikumar, B.1
Rubinsztein, D.C.2
-
27
-
-
2642586352
-
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
-
Ravikumar B., 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
-
28
-
-
18544400323
-
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
-
Scherzinger E., et al. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90 (1997) 549-558
-
(1997)
Cell
, vol.90
, pp. 549-558
-
-
Scherzinger, E.1
-
29
-
-
37849030901
-
Polyglutamine diseases: emerging concepts in pathogenesis and therapy.
-
Shao J., and Diamond M.I. Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum. Mol. Genet. 16 Spec No. 2 (2007) R115-R123
-
(2007)
Hum. Mol. Genet.
, vol.16
, Issue.Spec 2
-
-
Shao, J.1
Diamond, M.I.2
-
30
-
-
43549084329
-
ROCK and PRK-2 mediate the inhibitory effect of Y-27632 on polyglutamine aggregation
-
Shao J., et al. ROCK and PRK-2 mediate the inhibitory effect of Y-27632 on polyglutamine aggregation. FEBS Lett. 582 (2008) 1637-1642
-
(2008)
FEBS Lett.
, vol.582
, pp. 1637-1642
-
-
Shao, J.1
-
31
-
-
50249147874
-
Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation
-
Shao J., et al. Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation. Mol. Cell. Biol. 28 (2008) 5196-5208
-
(2008)
Mol. Cell. Biol.
, vol.28
, pp. 5196-5208
-
-
Shao, J.1
-
32
-
-
27644505297
-
Effects of fasudil in acute ischemic stroke: results of a prospective placebo-controlled double-blind trial
-
Shibuya M., et al. Effects of fasudil in acute ischemic stroke: results of a prospective placebo-controlled double-blind trial. J. Neurol. Sci. 238 (2005) 31-39
-
(2005)
J. Neurol. Sci.
, vol.238
, pp. 31-39
-
-
Shibuya, M.1
-
33
-
-
70350322713
-
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
-
Takahashi T., et al. Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum. Mol. Genet. (2007)
-
(2007)
Hum. Mol. Genet.
-
-
Takahashi, T.1
-
34
-
-
34548227453
-
Detection of polyglutamine protein oligomers in cells by fluorescence correlation spectroscopy
-
Takahashi Y., et al. Detection of polyglutamine protein oligomers in cells by fluorescence correlation spectroscopy. J. Biol. Chem. 282 (2007) 24039-24048
-
(2007)
J. Biol. Chem.
, vol.282
, pp. 24039-24048
-
-
Takahashi, Y.1
-
35
-
-
1642633757
-
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
-
Tanaka M., et al. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med. 10 (2004) 148-154
-
(2004)
Nat. Med.
, vol.10
, pp. 148-154
-
-
Tanaka, M.1
-
36
-
-
0030656619
-
Calcium sensitization of smooth muscle mediated by a Rho-associated protein kinase in hypertension
-
Uehata M., et al. Calcium sensitization of smooth muscle mediated by a Rho-associated protein kinase in hypertension. Nature 389 (1997) 990-994
-
(1997)
Nature
, vol.389
, pp. 990-994
-
-
Uehata, M.1
-
37
-
-
33644996796
-
Novel arylpyrazole compounds selectively modulate glucocorticoid receptor regulatory activity
-
Wang J.C., et al. Novel arylpyrazole compounds selectively modulate glucocorticoid receptor regulatory activity. Genes Dev. 20 (2006) 689-699
-
(2006)
Genes Dev.
, vol.20
, pp. 689-699
-
-
Wang, J.C.1
-
38
-
-
20044390015
-
A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo
-
Zhang X., 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
|