-
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
-
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, 971 (1993).
-
(1993)
Cell
, vol.72
, pp. 971
-
-
-
2
-
-
77349122826
-
CAG-repeat length and the age of onset in huntington disease (HD): A review and validation study of statistical approaches
-
Langbehn, D. R., Hayden, M. R. & Paulsen, J. S. CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches. Am. J. Med. Genet. B: Neuropsych. Genet. 153B, 397 (2010).
-
(2011)
Am. J. Med. Genet. B: Neuropsych. Genet
, vol.153
, pp. 397
-
-
Langbehn, D.R.1
Hayden, M.R.2
Paulsen, J.S.3
-
3
-
-
12144288251
-
Project T. U. S.-V. C. R. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset
-
Wexler, N. S. & Project, T. U. S.-V. C. R. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. PNAS 101, 3498 (2004).
-
(2004)
PNAS
, vol.101
, pp. 3498
-
-
Wexler, N.S.1
-
4
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
Yamamoto, A., Lucas, J. J. & Hen, R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101, 57 (2000).
-
(2000)
Cell
, vol.101
, pp. 57
-
-
Yamamoto, A.1
Lucas, J.J.2
Hen, R.3
-
5
-
-
20244378556
-
From the Cover: RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
-
Harper, S. Q. et al. From the Cover: RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. PNAS 102, 5820 (2005).
-
(2005)
PNAS
, vol.102
, pp. 5820
-
-
Harper, S.Q.1
-
6
-
-
33645104092
-
RAAV-mediated shRNA ameliorated neuropathology in Huntington disease model mouse
-
Machida, Y. et al. rAAV-mediated shRNA ameliorated neuropathology in Huntington disease model mouse. Biochem. Biophys. Res. Comm. 343, 190 (2006).
-
(2006)
Biochem. Biophys. Res. Comm
, vol.343
, pp. 190
-
-
Machida, Y.1
-
7
-
-
25144464388
-
Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice
-
Rodriguez-Lebron, E., Denovan-Wright, E. M., Nash, K., Lewin, A. S. & Mandel, R. J. 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, 618 (2005).
-
(2005)
Mol. Ther
, vol.12
, pp. 618
-
-
Rodriguez-Lebron, E.1
Denovan-Wright, E.M.2
Nash, K.3
Lewin, A.S.4
Mandel, R.J.5
-
8
-
-
36749033738
-
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
-
DiFiglia, M. et al. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. PNAS 104, 17204 (2007).
-
(2007)
PNAS
, vol.104
, pp. 17204
-
-
DiFiglia, M.1
-
9
-
-
26844494370
-
Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA
-
Wang, Y.-L. et al. Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA. Neurosci. R. 53, 241 (2005).
-
(2005)
Neurosci. R
, vol.53
, pp. 241
-
-
Wang, Y.-L.1
-
10
-
-
42549134402
-
AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease
-
Franich, N. R. et al. AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease. Mol. Ther. 16, 947 (2008).
-
(2008)
Mol. Ther
, vol.16
, pp. 947
-
-
Franich, N.R.1
-
11
-
-
65249131740
-
Sustained effects of nonallele-specific huntingtin silencing
-
Drouet, V. et al. Sustained effects of nonallele-specific huntingtin silencing. Ann. Neurol. 65, 276 (2009).
-
(2009)
Ann. Neurol
, vol.65
, pp. 276
-
-
Drouet, V.1
-
12
-
-
67349100160
-
Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in huntington's disease mice
-
Boudreau, R. L. et al. Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in huntington's disease mice. Mol. Ther. 17, 1053 (2009).
-
(2009)
Mol. Ther
, vol.17
, pp. 1053
-
-
Boudreau, R.L.1
-
13
-
-
84862663712
-
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis
-
Kordasiewicz, Holly B. et al. Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron 74, 1031 (2012).
-
(2012)
Neuron
, vol.74
, pp. 1031
-
-
Kordasiewicz Holly, B.1
-
14
-
-
84954299860
-
Neurobiology of Huntington's Disease: Applications to
-
edited by D C. Lo & R. E. Hughes, CRC Press, Boca Raton
-
Khoshnan, A., Southwell, A., Bugg, C., Ko, J. & Patterson, P. in Neurobiology of Huntington's Disease: Applications to Drug Discovery, edited by D. C. Lo & R. E. Hughes (CRC Press, Boca Raton, 2011).
-
(2011)
Drug Discovery
-
-
Khoshnan, A.1
Southwell, A.2
Bugg, C.3
Ko, J.4
Patterson, P.5
-
15
-
-
37049029416
-
R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice
-
Reiner, A. et al. R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice. J. Comp. Neurol. 505, 603 (2007).
-
(2007)
J. Comp. Neurol
, vol.505
, pp. 603
-
-
Reiner, A.1
-
16
-
-
82955237522
-
Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene /Allele-specific silencing of mutant huntingtin
-
Carroll, J. B. 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, 2178 (2011).
-
(2011)
Mol. Ther
, vol.19
, pp. 2178
-
-
Carroll, J.B.1
-
17
-
-
84871214247
-
A fully humanized transgenic mouse model of Huntington disease
-
Southwell, A. L. et al. A fully humanized transgenic mouse model of Huntington disease. Hum. Mol. Genet. 22, 18 (2013).
-
(2013)
Hum. Mol. Genet
, vol.22
, pp. 18
-
-
Southwell, A.L.1
-
18
-
-
70349103737
-
Single-step detection of mutant huntingtin in animal and human tissues: A bioassay for Huntington's disease
-
Weiss, A. et al. Single-step detection of mutant huntingtin in animal and human tissues: A bioassay for Huntington's disease. Anal. Biochem. 395, 8 (2009).
-
(2009)
Anal. Biochem
, vol.395
, pp. 8
-
-
Weiss, A.1
-
19
-
-
84901217827
-
Quantification assays for total and polyglutamine-expanded huntingtin proteins
-
Macdonald, D. et al. Quantification assays for total and polyglutamine-expanded huntingtin proteins, PLoS ONE 9, e96854 (2014).
-
(2014)
PLoS ONE
, vol.9
, pp. e96854
-
-
Macdonald, D.1
-
20
-
-
34250684995
-
High-sensitivity detection and quantitative analysis of native protein-protein interactions and multiprotein complexes by flow cytometry
-
Schrum, A. G. et al. High-sensitivity detection and quantitative analysis of native protein-protein interactions and multiprotein complexes by flow cytometry. Sci. Signal. 2007, pl2 (2007).
-
(2007)
Sci. Signal
, vol.2007
, pp. l2
-
-
Schrum, A.G.1
-
21
-
-
84866719263
-
IP-FCM measures physiologic protein-protein interactions modulated by signal transduction and smallmolecule drug inhibition
-
Smith, S. E. P. et al. IP-FCM measures physiologic protein-protein interactions modulated by signal transduction and smallmolecule drug inhibition. PLoS ONE 7, e45722 (2012).
-
(2012)
PLoS ONE
, vol.7
, pp. e45722
-
-
Smith, S.E.P.1
-
22
-
-
0035502934
-
New anti-huntingtin monoclonal antibodies: Implications for huntingtin conformation and its binding proteins
-
Ko, J., Ou, S. & Patterson, P. H. New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins. Brain Res. Bull. 56, 319 (2001).
-
(2001)
Brain Res. Bull
, vol.56
, pp. 319
-
-
Ko, J.1
Ou, S.2
Patterson, P.H.3
-
23
-
-
0037107151
-
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease
-
Wellington, C. L. et al. Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J. Neurosci. 22, 7862 (2002).
-
(2002)
J. Neurosci
, vol.22
, pp. 7862
-
-
Wellington, C.L.1
-
24
-
-
0029055717
-
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
-
Nasir, J. et al. Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81, 811 (1995).
-
(1995)
Cell
, vol.81
, pp. 811
-
-
Nasir, J.1
-
25
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
-
Zeitlin, S., Liu, J. P., Chapman, D. L., Papaioannou, V. E. & Efstratiadis, A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nat. genet. 11, 155 (1995).
-
(1995)
Nat. Genet
, vol.11
, pp. 155
-
-
Zeitlin, S.1
Liu, J.P.2
Chapman, D.L.3
Papaioannou, V.E.4
Efstratiadis, A.5
-
26
-
-
46749157501
-
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
-
Gray, M. et al. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J. Neurosci. 28, 6182 (2008).
-
(2008)
J. Neurosci
, vol.28
, pp. 6182
-
-
Gray, M.1
-
27
-
-
0032842478
-
Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety
-
Tronche, F. et al. Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety. Nat. Genet. 23, 99 (1999).
-
(1999)
Nat. Genet
, vol.23
, pp. 99
-
-
Tronche, F.1
-
28
-
-
67449094981
-
Mutant huntingtin n-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells
-
Ratovitski, T. et al. Mutant huntingtin n-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells. J. Biol. Chem. 284, 10855 (2009).
-
(2009)
J. Biol. Chem
, vol.284
, pp. 10855
-
-
Ratovitski, T.1
-
29
-
-
0035869544
-
Tissue-specific proteolysis of huntingtin (htt) in human brain: Evidence of enhanced levels of N-and C-terminal htt fragments in Huntington's disease striatum
-
Mende-Mueller, L. M., Toneff, T., Hwang, S.-R., Chesselet, M.-F. & Hook, V. Y. H. Tissue-specific proteolysis of huntingtin (htt) in human brain: evidence of enhanced levels of N-and C-terminal htt fragments in Huntington's disease striatum. J. Neurosci. 21, 1830 (2001).
-
(2001)
J. Neurosci
, vol.21
, pp. 1830
-
-
Mende-Mueller, L.M.1
Toneff, T.2
Hwang, S.-R.3
Chesselet, M.-F.4
Hook, V.Y.H.5
-
30
-
-
10744227174
-
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
-
Slow, E. J. et al. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 12, 1555 (2003).
-
(2003)
Hum. Mol. Genet
, vol.12
, pp. 1555
-
-
Slow, E.J.1
-
31
-
-
84927177598
-
In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides
-
Southwell, A. L. et al. In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides. Mol. Ther. 22, 2093 (2014).
-
(2014)
Mol. Ther
, vol.22
, pp. 2093
-
-
Southwell, A.L.1
-
32
-
-
60849093185
-
Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease
-
Graham, R. K. et al. Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. J. Neurosci. 29, 2193 (2009).
-
(2009)
J. Neurosci
, vol.29
, pp. 2193
-
-
Graham, R.K.1
-
33
-
-
80055116623
-
IP-FCM: Immunoprecipitation detected by flow cytometry
-
Davis, T. R. & Schrum, A. G. IP-FCM: Immunoprecipitation detected by flow cytometry. J. Vis. Exp. 46, e2066 (2010).
-
(2011)
J. Vis. Exp
, vol.46
, pp. e2066
-
-
Davis, T.R.1
Schrum, A.G.2
|