-
3
-
-
0025290717
-
Huntington's disease in Venezuela: Seven years of follow-up on symptomatic and asymptomatic individuals
-
Penney Jr JB, Young AB, Shoulson I, et al. Huntington's disease in Venezuela: seven years of follow-up on symptomatic and asymptomatic individuals. Mov Disord 1990;5:93-99.
-
(1990)
Mov Disord
, vol.5
, pp. 93-99
-
-
Penney Jr., J.B.1
Young, A.B.2
Shoulson, I.3
-
4
-
-
0023935740
-
Differential cognitive impairment in Alzheimer disease and Huntington's disease
-
Brandt J, Folstein SE, Folstein MF. Differential cognitive impairment in Alzheimer disease and Huntington's disease. Ann Neurol 1988;23:555-561.
-
(1988)
Ann Neurol
, vol.23
, pp. 555-561
-
-
Brandt, J.1
Folstein, S.E.2
Folstein, M.F.3
-
7
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi HY, Orr HT. Glutamine repeats and neurodegeneration. Annu Rev Neurosci 2000;23:217-247.
-
(2000)
Annu Rev Neurosci
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
-
8
-
-
0030868770
-
Reviews in molecular medicine: Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA)
-
Ross CA, Margolis RL, Rosenblatt A, Ranen NG, Becher MW, Aylward E. Reviews in molecular medicine: Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA). Medicine 1997;76:305-338.
-
(1997)
Medicine
, vol.76
, pp. 305-338
-
-
Ross, C.A.1
Margolis, R.L.2
Rosenblatt, A.3
Ranen, N.G.4
Becher, M.W.5
Aylward, E.6
-
9
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
-
Group HDCR. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72:971-983.
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
10
-
-
0030948716
-
The genetic defect causing Huntington's disease: Repeated in other contexts?
-
Gusella JF, Persichetti F, MacDonald ME. The genetic defect causing Huntington's disease: repeated in other contexts? Mol Med 1997;3:238-246.
-
(1997)
Mol Med
, vol.3
, pp. 238-246
-
-
Gusella, J.F.1
Persichetti, F.2
MacDonald, M.E.3
-
11
-
-
0029089172
-
When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases
-
Ross CA. When more is less: pathogenesis of glutamine repeat neurodegenerative diseases. Neuron 1995;15:493-496.
-
(1995)
Neuron
, vol.15
, pp. 493-496
-
-
Ross, C.A.1
-
12
-
-
0028260436
-
Structure and expression of the Huntington's disease gene: Evidence against simple inactivation due to an expanded CAG repeat
-
Ambrose CM, Duyao MP, Barnes G, et al. Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat. Somat Cell Mol Genet 1994;20:27-28.
-
(1994)
Somat Cell Mol Genet
, vol.20
, pp. 27-28
-
-
Ambrose, C.M.1
Duyao, M.P.2
Barnes, G.3
-
13
-
-
0029997090
-
Phenotypic characterization of individuals with 30-40 CAG repeats and apparently normal elderly individuals with 36-39 repeats
-
Rubinsztein DC, Leggo J, Coles R, et al. Phenotypic characterization of individuals with 30-40 CAG repeats and apparently normal elderly individuals with 36-39 repeats. Am J Hum Genet 1996;59:16-22.
-
(1996)
Am J Hum Genet
, vol.59
, pp. 16-22
-
-
Rubinsztein, D.C.1
Leggo, J.2
Coles, R.3
-
14
-
-
0029908301
-
Anticipation - An old idea in new genes
-
McInnis MG. Anticipation - an old idea in new genes. Am J Hum Genet 1996;59:973-979.
-
(1996)
Am J Hum Genet
, vol.59
, pp. 973-979
-
-
McInnis, M.G.1
-
15
-
-
0027240431
-
Trinucleotide repeat length instability and age of onset in Huntington's disease
-
Duyao M, Ambrose C, Myers R, et al. Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet 1993;4:387-392.
-
(1993)
Nat Genet
, vol.4
, pp. 387-392
-
-
Duyao, M.1
Ambrose, C.2
Myers, R.3
-
16
-
-
0027377151
-
Correlation between the onset of Huntington's disease and length of the trinucleotide repeat in IT-15
-
Stine OC, Pleasant N, Franz ML, Abbott MH, Folstein SE, Ross CA. Correlation between the onset of Huntington's disease and length of the trinucleotide repeat in IT-15. Hum Mol Genet 1993;2:1547-1549.
-
(1993)
Hum Mol Genet
, vol.2
, pp. 1547-1549
-
-
Stine, O.C.1
Pleasant, N.2
Franz, M.L.3
Abbott, M.H.4
Folstein, S.E.5
Ross, C.A.6
-
17
-
-
0029130324
-
Anticipation and instability of (CAG)n repeats in IT-15 in parent-offspring pairs with Huntington's disease
-
Ranen NG, Stine OC, Abbott MH, et al. Anticipation and instability of (CAG)n repeats in IT-15 in parent-offspring pairs with Huntington's disease. Am J Hum Genet 1995;57:593-602.
-
(1995)
Am J Hum Genet
, vol.57
, pp. 593-602
-
-
Ranen, N.G.1
Stine, O.C.2
Abbott, M.H.3
-
18
-
-
0027176364
-
The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease
-
Andrew SE, Goldberg YP, Kremer B, et al. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease. Nat Genet 1993;4:398-403.
-
(1993)
Nat Genet
, vol.4
, pp. 398-403
-
-
Andrew, S.E.1
Goldberg, Y.P.2
Kremer, B.3
-
19
-
-
0028985055
-
Diagnosis of 'sporadic' Huntington's disease
-
Durr A, Dode C, Hahn V, et al. Diagnosis of 'sporadic' Huntington's disease. J Neurol Sci 1995;129:51-55.
-
(1995)
J Neurol Sci
, vol.129
, pp. 51-55
-
-
Durr, A.1
Dode, C.2
Hahn, V.3
-
20
-
-
0031037349
-
Longitudinal change in basal ganglia volume in patients with Huntington's disease
-
Aylward EH, Li Q, Stine OC, et al. Longitudinal change in basal ganglia volume in patients with Huntington's disease. Neurology 1997;48:394-399.
-
(1997)
Neurology
, vol.48
, pp. 394-399
-
-
Aylward, E.H.1
Li, Q.2
Stine, O.C.3
-
21
-
-
0028099274
-
Trinucleotide repeat length and progression of illness in Huntington's disease
-
Kieburtz K, MacDonald M, Shih C, et al. Trinucleotide repeat length and progression of illness in Huntington's disease. J Med Genet 1994;31:872-874.
-
(1994)
J Med Genet
, vol.31
, pp. 872-874
-
-
Kieburtz, K.1
MacDonald, M.2
Shih, C.3
-
22
-
-
0030069713
-
Relationship between trinucleotide repeats and neuropathological changes in Huntington disease
-
Furtado S, Suchowersky O, Rewcastle B, Graham L, Klimek ML, Garber A. Relationship between trinucleotide repeats and neuropathological changes in Huntington disease. Ann Neurol 1996;39:132-136.
-
(1996)
Ann Neurol
, vol.39
, pp. 132-136
-
-
Furtado, S.1
Suchowersky, O.2
Rewcastle, B.3
Graham, L.4
Klimek, M.L.5
Garber, A.6
-
23
-
-
0022343684
-
Selective sparing of a class of striatal neurons in Huntington's disease
-
Ferrante RJ, Kowall NW, Beal MF, Richardson Jr EP, Bird ED, Martin JB. Selective sparing of a class of striatal neurons in Huntington's disease. Science 1985;230:561-563.
-
(1985)
Science
, vol.230
, pp. 561-563
-
-
Ferrante, R.J.1
Kowall, N.W.2
Beal, M.F.3
Richardson Jr., E.P.4
Bird, E.D.5
Martin, J.B.6
-
24
-
-
0026717486
-
Preferential loss of striato external pallidal projection neurons in presymptomatic Huntington' s disease
-
Albin RL, Reiner A, Anderson KD, et al. Preferential loss of striato external pallidal projection neurons in presymptomatic Huntington' s disease. Ann Neurol 1992;31:425-430.
-
(1992)
Ann Neurol
, vol.31
, pp. 425-430
-
-
Albin, R.L.1
Reiner, A.2
Anderson, K.D.3
-
26
-
-
0022395922
-
Neuropathological classification of Huntington' s disease
-
Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP. Neuropathological classification of Huntington' s disease. J Neuropathol Exp Neurol 1985;44:559-577.
-
(1985)
J Neuropathol Exp Neurol
, vol.44
, pp. 559-577
-
-
Vonsattel, J.P.1
Myers, R.H.2
Stevens, T.J.3
Ferrante, R.J.4
Bird, E.D.5
Richardson, E.P.6
-
27
-
-
0023865274
-
Clinical and neuropathologic assessment of severity in Huntington disease
-
Myers RH, Vonsattel J-P, Stevens TJ, et al. Clinical and neuropathologic assessment of severity in Huntington disease. Neurology 1988;38:341-347.
-
(1988)
Neurology
, vol.38
, pp. 341-347
-
-
Myers, R.H.1
Vonsattel, J.-P.2
Stevens, T.J.3
-
28
-
-
0025885733
-
Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease
-
Hedreen JC, Peyser CE, Folstein SE, Ross CA. Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease. Neurosci Lett 1991;133:257-261.
-
(1991)
Neurosci Lett
, vol.133
, pp. 257-261
-
-
Hedreen, J.C.1
Peyser, C.E.2
Folstein, S.E.3
Ross, C.A.4
-
29
-
-
0021921929
-
A Golgi study of the human neostriatum: Neurons and afferent fibers
-
Graveland GA, Williams RS, DiFiglia M. A Golgi study of the human neostriatum: neurons and afferent fibers. J Comp Neurol 1985;234:317-333.
-
(1985)
J Comp Neurol
, vol.234
, pp. 317-333
-
-
Graveland, G.A.1
Williams, R.S.2
DiFiglia, M.3
-
30
-
-
0027377217
-
Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington' s disease: A quantitative Golgi study
-
Sotrel A, Williams RS, Kaufmann WE, Myers RH. Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington' s disease: a quantitative Golgi study. Neurology 1993;43:2088-2986.
-
(1993)
Neurology
, vol.43
, pp. 2088-2986
-
-
Sotrel, A.1
Williams, R.S.2
Kaufmann, W.E.3
Myers, R.H.4
-
31
-
-
0021891247
-
Huntington's disease: Two families with differing clinical features show linkage to the G8 probe
-
Folstein SE, Phillips III JA, Meyers DA, et al. Huntington's disease: two families with differing clinical features show linkage to the G8 probe. Science 1985;229:776-779.
-
(1985)
Science
, vol.229
, pp. 776-779
-
-
Folstein, S.E.1
Phillips III, J.A.2
Meyers, D.A.3
-
32
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuntes in brain
-
DiFiglia M, Sapp E, Chase KO, et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuntes in brain. Science 1997;277:1990-1993.
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
-
33
-
-
0031918640
-
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length
-
Becher MW, Kotzuk JA, Sharp AH, et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998;4:387-397.
-
(1998)
Neurobiol Dis
, vol.4
, pp. 387-397
-
-
Becher, M.W.1
Kotzuk, J.A.2
Sharp, A.H.3
-
34
-
-
0342454322
-
Pathogenesis of polyglutamine neurodegenerative diseases: Towards a unifying mechanism
-
Wells RD, Warren ST, editors. San Diego, CA: Academic Press
-
Ross CA, Margolis RL, Becher MW, Wood JD, Engelender S, Sharp AH. Pathogenesis of polyglutamine neurodegenerative diseases: towards a unifying mechanism. In: Wells RD, Warren ST, editors. Genetic instabilities and hereditary neurological diseases, San Diego, CA: Academic Press, 1998.
-
(1998)
Genetic Instabilities and Hereditary Neurological Diseases
-
-
Ross, C.A.1
Margolis, R.L.2
Becher, M.W.3
Wood, J.D.4
Engelender, S.5
Sharp, A.H.6
-
35
-
-
18544410106
-
Formation of neuronal intranuclear inclusions (Nil) underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies SW, Turmaine M, Cozens BA, et al. Formation of neuronal intranuclear inclusions (Nil) underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997;90:537-548.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
-
36
-
-
0023115076
-
Homozygotes for Huntington's disease
-
Wexler NS, Young AB, Tanzi RE, et al. Homozygotes for Huntington's disease. Nature 1987;326:194-197.
-
(1987)
Nature
, vol.326
, pp. 194-197
-
-
Wexler, N.S.1
Young, A.B.2
Tanzi, R.E.3
-
37
-
-
0033525036
-
Molecular and cellular genetics of fragile X syndrome
-
Kaufmann WE, Reiss AL. Molecular and cellular genetics of fragile X syndrome. Am J Med Genet 1999;88:11-24.
-
(1999)
Am J Med Genet
, vol.88
, pp. 11-24
-
-
Kaufmann, W.E.1
Reiss, A.L.2
-
38
-
-
0030613177
-
Huntingtin is required for neurogenesis and is not impaired by the Huntington' s disease CAG expansion
-
White JK, Auerbach W, Duyao MP, et al. Huntingtin is required for neurogenesis and is not impaired by the Huntington' s disease CAG expansion. Nat Genet 1997;17:404-410.
-
(1997)
Nat Genet
, vol.17
, pp. 404-410
-
-
White, J.K.1
Auerbach, W.2
Duyao, M.P.3
-
39
-
-
0022446150
-
Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
-
Beal MF, Kowall NW, Ellison DZW, Mazurek MF, Swartz KJ, Martin JB. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature 1986;321:168-171.
-
(1986)
Nature
, vol.321
, pp. 168-171
-
-
Beal, M.F.1
Kowall, N.W.2
Ellison, D.Z.W.3
Mazurek, M.F.4
Swartz, K.J.5
Martin, J.B.6
-
40
-
-
0027433553
-
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid
-
Beal MF, Brouillet E, Jenkins B, et al. Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid. J Neurosci 1993;13:4181-4192.
-
(1993)
J Neurosci
, vol.13
, pp. 4181-4192
-
-
Beal, M.F.1
Brouillet, E.2
Jenkins, B.3
-
41
-
-
0033373421
-
Bioenergetics in Huntington's disease
-
Grunewald T, Beal MF. Bioenergetics in Huntington's disease. Ann N Y Acad Sci 1999;893:203-213.
-
(1999)
Ann N Y Acad Sci
, vol.893
, pp. 203-213
-
-
Grunewald, T.1
Beal, M.F.2
-
43
-
-
0033938117
-
Life and death decisions: Regulation of apoptosis by proteolysis of signaling molecules
-
Utz PJ, Anderson P. Life and death decisions: regulation of apoptosis by proteolysis of signaling molecules. Cell Death Differ 2000;7:589-602.
-
(2000)
Cell Death Differ
, vol.7
, pp. 589-602
-
-
Utz, P.J.1
Anderson, P.2
-
44
-
-
0034051284
-
Mechanisms of neurodegenerative disorders: Part 2: control of cell death
-
Wolozin B, Behl C. Mechanisms of neurodegenerative disorders: part 2: control of cell death. Arch Neurol 2000;57:801-804.
-
(2000)
Arch Neurol
, vol.57
, pp. 801-804
-
-
Wolozin, B.1
Behl, C.2
-
45
-
-
0031044805
-
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10
-
Koroshetz WJ, Jenkins BG, Rosen BR, Beal MF. Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol 1997;41:160-165.
-
(1997)
Ann Neurol
, vol.41
, pp. 160-165
-
-
Koroshetz, W.J.1
Jenkins, B.G.2
Rosen, B.R.3
Beal, M.F.4
-
46
-
-
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;41:646-653.
-
(1997)
Ann Neurol
, vol.41
, pp. 646-653
-
-
Browne, S.E.1
Bowling, A.C.2
MacGarvey, U.3
-
47
-
-
0029875381
-
Mitochondrial defect in Huntington's disease caudate nucleus
-
Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AHV. Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol 1996;39:385-389.
-
(1996)
Ann Neurol
, vol.39
, pp. 385-389
-
-
Gu, M.1
Gash, M.T.2
Mann, V.M.3
Javoy-Agid, F.4
Cooper, J.M.5
Schapira, A.H.V.6
-
48
-
-
0029034511
-
Widespread expression of the Huntington's disease gene (IT15) protein product
-
Sharp AH, Loev SJ, Schilling G, et al. Widespread expression of the Huntington's disease gene (IT15) protein product. Neuron 1995;14:1065-1074.
-
(1995)
Neuron
, vol.14
, pp. 1065-1074
-
-
Sharp, A.H.1
Loev, S.J.2
Schilling, G.3
-
49
-
-
0028989602
-
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
-
DiFiglia M, Sapp E, Chase K, et al. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 1995;14:1075-1081.
-
(1995)
Neuron
, vol.14
, pp. 1075-1081
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.3
-
50
-
-
0028972448
-
Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias
-
Trottier Y, Lutz Y, Stevanin G, et al. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 1995;378:403-406.
-
(1995)
Nature
, vol.378
, pp. 403-406
-
-
Trottier, Y.1
Lutz, Y.2
Stevanin, G.3
-
51
-
-
1842336432
-
Wild-type and mutant huntingtin localize to the golgi complex and to vesicles in the peripheral cytoplasm in fibroblasts of control and HD patients
-
Velier J, Schwarz C, Young C, et al. Wild-type and mutant huntingtin localize to the golgi complex and to vesicles in the peripheral cytoplasm in fibroblasts of control and HD patients. Soc Neurosci Abstracts 1996;22:226.
-
(1996)
Soc Neurosci Abstracts
, vol.22
, pp. 226
-
-
Velier, J.1
Schwarz, C.2
Young, C.3
-
53
-
-
0029664992
-
Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH
-
Burke JR, Enghild JJ, Martin ME, et al. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med 1996;347:350.
-
(1996)
Nat Med
, vol.347
, pp. 350
-
-
Burke, J.R.1
Enghild, J.J.2
Martin, M.E.3
-
54
-
-
0032833981
-
Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin
-
Boutell JM, Thomas P, Neal JW, et al. Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum Mol Genet 1999;8:1647-1655.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 1647-1655
-
-
Boutell, J.M.1
Thomas, P.2
Neal, J.W.3
-
55
-
-
0027507667
-
Human genetic diseases due to codon reiteration: Relationship to an evolutionary mechanism
-
Green H. Human genetic diseases due to codon reiteration: relationship to an evolutionary mechanism. Cell 1993;74:955-956.
-
(1993)
Cell
, vol.74
, pp. 955-956
-
-
Green, H.1
-
56
-
-
0030840917
-
cDNAs with long CAG trinucleotide repeats from human brain
-
Margolis RL, Abraham MA, Gatchell SB, et al. cDNAs with long CAG trinucleotide repeats from human brain. Hum Genet 1997;100:114-122.
-
(1997)
Hum Genet
, vol.100
, pp. 114-122
-
-
Margolis, R.L.1
Abraham, M.A.2
Gatchell, S.B.3
-
57
-
-
0027988041
-
Polar zippers: Their role in human disease
-
London: Cambridge University Press
-
Perutz M. Polar zippers: their role in human disease. Protein science, 3rd ed. London: Cambridge University Press, 1994. pp. 1629-1637.
-
(1994)
Protein Science, 3rd Ed.
, pp. 1629-1637
-
-
Perutz, M.1
-
58
-
-
0029856046
-
Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: Relevance to diseases of the nervous system
-
Kahlem P, Terre C, Green H, Djian P. Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system. Proc Natl Acad Sci USA 1996;93:14580-14585.
-
(1996)
Proc Natl Acad Sci USA
, vol.93
, pp. 14580-14585
-
-
Kahlem, P.1
Terre, C.2
Green, H.3
Djian, P.4
-
60
-
-
0033551063
-
Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: Implications for Huntington' s disease pathology
-
Scherzinger E, Sittler A, Schweiger K, et al. Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington' s disease pathology. Proc Natl Acad Sci USA 1999;96:4604-4609.
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 4604-4609
-
-
Scherzinger, E.1
Sittler, A.2
Schweiger, K.3
-
61
-
-
0032847708
-
Nuclear targeting of mutant huntingtin increases toxicity
-
Peters MF, Nucifora Jr FC, Kushi J, et al. Nuclear targeting of mutant huntingtin increases toxicity. Mol Cell Neurosci 1999;14:121-128.
-
(1999)
Mol Cell Neurosci
, vol.14
, pp. 121-128
-
-
Peters, M.F.1
Nucifora Jr., F.C.2
Kushi, J.3
-
62
-
-
0031680014
-
A cellular model that recapitulates major pathogenic steps of Huntington's disease
-
Lunkes A, Mandel JL. A cellular model that recapitulates major pathogenic steps of Huntington's disease. Hum Mol Genet 1998;7:1355-1361.
-
(1998)
Hum Mol Genet
, vol.7
, pp. 1355-1361
-
-
Lunkes, A.1
Mandel, J.L.2
-
63
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
Saudou F, Finkbeiner S, Devys D, Greenberg ME. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998;95:55-66.
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
64
-
-
0033556160
-
Generation of neuronal intranuclear inclusions by polyglutamine-GFP: Analysis of inclusion clearance and toxicity as a function of polyglutamine length
-
Moulder KL, Onodera O, Burke JR, Strittmatter WJ, Johnson Jr EM. Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length. J Neurosci 1999;19:705-715.
-
(1999)
J Neurosci
, vol.19
, pp. 705-715
-
-
Moulder, K.L.1
Onodera, O.2
Burke, J.R.3
Strittmatter, W.J.4
Johnson Jr., E.M.5
-
65
-
-
0032101287
-
The influence of huntingtin protein size on nuclear localization and cellular toxicity
-
Hackam AS, Singaraja R, Wellington CL, et al. The influence of huntingtin protein size on nuclear localization and cellular toxicity. J Cell Biol 1998;141:1097-1105.
-
(1998)
J Cell Biol
, vol.141
, pp. 1097-1105
-
-
Hackam, A.S.1
Singaraja, R.2
Wellington, C.L.3
-
66
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale D, Hackam A, Wieczorek A, et al. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet 1998;18:150-154.
-
(1998)
Nat Genet
, vol.18
, pp. 150-154
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
-
67
-
-
7144253143
-
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture
-
Cooper JK, Schilling G, Peters MF, et al. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum Mol Genet 1998;7:783-790.
-
(1998)
Hum Mol Genet
, vol.7
, pp. 783-790
-
-
Cooper, J.K.1
Schilling, G.2
Peters, M.F.3
-
68
-
-
0033081766
-
Mutant huntingtin expression in clonal striatal cells: Dissociation of inclusion formation and neuronal survival by caspase inhibition
-
Kim M, Lee HS, LaForet G, et al. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J Neurosci 1999;19:964-973.
-
(1999)
J Neurosci
, vol.19
, pp. 964-973
-
-
Kim, M.1
Lee, H.S.2
LaForet, G.3
-
69
-
-
0032811511
-
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
-
Li H, Li SH, Cheng AL, Mangiarini L, Bates GP, Li XJ. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum Mol Genet 1999;8:1227-1236.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 1227-1236
-
-
Li, H.1
Li, S.H.2
Cheng, A.L.3
Mangiarini, L.4
Bates, G.P.5
Li, X.J.6
-
70
-
-
0032502715
-
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
-
Wellington CL, Ellerby LM, Hackam AS, et al. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem 1998;273:9158-9167.
-
(1998)
J Biol Chem
, vol.273
, pp. 9158-9167
-
-
Wellington, C.L.1
Ellerby, L.M.2
Hackam, A.S.3
-
71
-
-
0033953015
-
Caspases and neurodegeneration: On the cutting edge of new therapeutic approaches
-
Wellington CL, Hayden MR. Caspases and neurodegeneration: on the cutting edge of new therapeutic approaches. Clin Genet 2000;57:1-10.
-
(2000)
Clin Genet
, vol.57
, pp. 1-10
-
-
Wellington, C.L.1
Hayden, M.R.2
-
72
-
-
0034733607
-
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and non-neuronal cells
-
Wellington CL, Singaraja R, Ellerby L, et al. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and non-neuronal cells. J Biol Chem 2000;275:19831-19838.
-
(2000)
J Biol Chem
, vol.275
, pp. 19831-19838
-
-
Wellington, C.L.1
Singaraja, R.2
Ellerby, L.3
-
73
-
-
0033103523
-
Caspase-8 is required for cell death induced by expanded polyglutamine repeats
-
Sanchez I, Xu CJ, Juo P, Kakizaka A, Blenis J, Yuan J. Caspase-8 is required for cell death induced by expanded polyglutamine repeats. Neuron 1999;22:623-633.
-
(1999)
Neuron
, vol.22
, pp. 623-633
-
-
Sanchez, I.1
Xu, C.J.2
Juo, P.3
Kakizaka, A.4
Blenis, J.5
Yuan, J.6
-
74
-
-
0033605746
-
Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity
-
Ellerby LM, Andrusiak RL, Wellington CL, et al. Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity. J Biol Chem 1999;274:8730-8736.
-
(1999)
J Biol Chem
, vol.274
, pp. 8730-8736
-
-
Ellerby, L.M.1
Andrusiak, R.L.2
Wellington, C.L.3
-
75
-
-
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, Sathasivam S, Seller M, 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 1996;87:493-506.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, S.2
Seller, M.3
-
76
-
-
0033560924
-
Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
-
Carter RJ, Lione LA, Humby T, et al. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 1999;19:3248-3257.
-
(1999)
J Neurosci
, vol.19
, pp. 3248-3257
-
-
Carter, R.J.1
Lione, L.A.2
Humby, T.3
-
77
-
-
0034234519
-
Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation
-
Murphy KP, Carter RJ, Lione LA, et al. Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J Neurosci 2000;20:5115-5123.
-
(2000)
J Neurosci
, vol.20
, pp. 5115-5123
-
-
Murphy, K.P.1
Carter, R.J.2
Lione, L.A.3
-
78
-
-
0033054555
-
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
-
Schilling G, Becher MW, Sharp AH, et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 1999;8:397-407.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 397-407
-
-
Schilling, G.1
Becher, M.W.2
Sharp, A.H.3
-
79
-
-
17344367977
-
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
-
Reddy PH, Williams M, Charles V, et al. Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. Nat Genet 1998;20:198-202.
-
(1998)
Nat Genet
, vol.20
, pp. 198-202
-
-
Reddy, P.H.1
Williams, M.2
Charles, V.3
-
80
-
-
0033136692
-
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
-
Hodgson JG, Agopyan N, Gutekunst CA, Leavitt BR, LePiane F, Singaraja R. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 1999;23:181-192.
-
(1999)
Neuron
, vol.23
, pp. 181-192
-
-
Hodgson, J.G.1
Agopyan, N.2
Gutekunst, C.A.3
Leavitt, B.R.4
LePiane, F.5
Singaraja, R.6
-
81
-
-
0034163497
-
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
-
Wheeler VC, White JK, Gutekunst CA, et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet 2000;9:503-513.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 503-513
-
-
Wheeler, V.C.1
White, J.K.2
Gutekunst, C.A.3
-
82
-
-
0033848272
-
Expression of Huntingtin-associated protein-1 in neuronal cells implicates a role in neuritic growth
-
Li SH, Li H, Torre ER, Li XJ. Expression of Huntingtin-associated protein-1 in neuronal cells implicates a role in neuritic growth. Mol Cell Neurosci 2000;16:168-183.
-
(2000)
Mol Cell Neurosci
, vol.16
, pp. 168-183
-
-
Li, S.H.1
Li, H.2
Torre, E.R.3
Li, X.J.4
-
83
-
-
0034737299
-
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
-
Yamamoto A, Lucas JJ, Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000;101:57-66.
-
(2000)
Cell
, vol.101
, pp. 57-66
-
-
Yamamoto, A.1
Lucas, J.J.2
Hen, R.3
-
84
-
-
18544392423
-
Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila
-
Warrick JM, Paulson HL, Gray-Board GL, et al. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell 1998;93:939-949.
-
(1998)
Cell
, vol.93
, pp. 939-949
-
-
Warrick, J.M.1
Paulson, H.L.2
Gray-Board, G.L.3
-
85
-
-
0032168160
-
Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons
-
Jackson GR, Salecker I, Dong X, et al. Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron 1998;21:633-642.
-
(1998)
Neuron
, vol.21
, pp. 633-642
-
-
Jackson, G.R.1
Salecker, I.2
Dong, X.3
-
86
-
-
0034629073
-
Genetic suppression of polyglutamine toxicity in Drosophila
-
Kazemi-Esfarjani P, Benzer S. Genetic suppression of polyglutamine toxicity in Drosophila. Science 2000;287:1837-1840.
-
(2000)
Science
, vol.287
, pp. 1837-1840
-
-
Kazemi-Esfarjani, P.1
Benzer, S.2
-
87
-
-
0032727617
-
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
-
Warrick JM, Chan HY, Gray-Board GL, Chai Y, Paulson HL, Bonini NM. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat Genet 1999;23:425-428.
-
(1999)
Nat Genet
, vol.23
, pp. 425-428
-
-
Warrick, J.M.1
Chan, H.Y.2
Gray-Board, G.L.3
Chai, Y.4
Paulson, H.L.5
Bonini, N.M.6
-
88
-
-
0033524413
-
Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron
-
Faber PW, Alter JR, MacDonald ME, Hart AC. Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc Natl Acad Sci USA 1999;96:179-184.
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 179-184
-
-
Faber, P.W.1
Alter, J.R.2
MacDonald, M.E.3
Hart, A.C.4
-
89
-
-
0034705224
-
Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans
-
Satyal SH, Schmidt E, Kitagawa K, et al. Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proc Natl Acad Sci USA 2000;97:5750-5755.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 5750-5755
-
-
Satyal, S.H.1
Schmidt, E.2
Kitagawa, K.3
-
90
-
-
0024556360
-
A controlled clinical trial of baclofen as protective therapy in early Huntington's disease
-
Shoulson I, Odoroff C, Oakes D, et al. A controlled clinical trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol 1989;25:252-259.
-
(1989)
Ann Neurol
, vol.25
, pp. 252-259
-
-
Shoulson, I.1
Odoroff, C.2
Oakes, D.3
-
91
-
-
9244263519
-
A controlled trial of remacemide hydrochloride in Huntington's disease
-
Kieburtz K, Feigin A, McDermott M, et al. A controlled trial of remacemide hydrochloride in Huntington's disease. Mov Disord 1996;11:273-277.
-
(1996)
Mov Disord
, vol.11
, pp. 273-277
-
-
Kieburtz, K.1
Feigin, A.2
McDermott, M.3
-
92
-
-
0029988363
-
Assessment of Coenzyme Q10 tolerability in Huntington's disease
-
Feigin A, Keiburtz K, Como P, et al. Assessment of Coenzyme Q10 tolerability in Huntington's disease. Mov Disord 1996;11:321-323.
-
(1996)
Mov Disord
, vol.11
, pp. 321-323
-
-
Feigin, A.1
Keiburtz, K.2
Como, P.3
-
93
-
-
9544255791
-
A controlled trial of idebenone in Huntington's disease
-
Ranen NG, Peyser CE, Coyle J, et al. A controlled trial of idebenone in Huntington's disease. Mov Disord 1996;11:549-554.
-
(1996)
Mov Disord
, vol.11
, pp. 549-554
-
-
Ranen, N.G.1
Peyser, C.E.2
Coyle, J.3
-
94
-
-
0028856571
-
Trial of d-alpha-tocopherol in Huntington's disease
-
Peyser CE, Folstein MF, Chase GA, et al. Trial of d-alpha-tocopherol in Huntington's disease. Am J Psychiatry 1995;152:1771-1775.
-
(1995)
Am J Psychiatry
, vol.152
, pp. 1771-1775
-
-
Peyser, C.E.1
Folstein, M.F.2
Chase, G.A.3
-
95
-
-
0034612220
-
Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: Implications for Huntington's disease therapy
-
Heiser V, Scherzinger E, Boeddrich A, et al. Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy. Proc Natl Acad Sci USA 2000;97:6739-6744.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 6739-6744
-
-
Heiser, V.1
Scherzinger, E.2
Boeddrich, A.3
-
96
-
-
0034608868
-
Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
-
Muchowski PJ, Schaffar G, Sittler A, Wanker EE, Hayer-Hartl MK, Hartl FU. Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proc Natl Acad Sci USA 2000;97:7841-7846.
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 7841-7846
-
-
Muchowski, P.J.1
Schaffar, G.2
Sittler, A.3
Wanker, E.E.4
Hayer-Hartl, M.K.5
Hartl, F.U.6
-
97
-
-
0033587128
-
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
-
Ona VO, Li M, Vonsattel JP, Andrews LJ, et al. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature 1999;399:263-267.
-
(1999)
Nature
, vol.399
, pp. 263-267
-
-
Ona, V.O.1
Li, M.2
Vonsattel, J.P.3
Andrews, L.J.4
-
98
-
-
0034660457
-
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
-
Ferrante RJ, Andreassen OA, Jenkins BG, et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci 2000;20:4389-4397.
-
(2000)
J Neurosci
, vol.20
, pp. 4389-4397
-
-
Ferrante, R.J.1
Andreassen, O.A.2
Jenkins, B.G.3
-
99
-
-
0033912716
-
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
-
Chen M, Ona VO, Li M, et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med 2000;6:797-801.
-
(2000)
Nat Med
, vol.6
, pp. 797-801
-
-
Chen, M.1
Ona, V.O.2
Li, M.3
-
100
-
-
0034643362
-
Delaying the onset of Huntington's in mice
-
van Dellen A, Blakemore C, Deacon R, York D, Hannan AJ. Delaying the onset of Huntington's in mice. Nature 2000;404:721-722.
-
(2000)
Nature
, vol.404
, pp. 721-722
-
-
Van Dellen, A.1
Blakemore, C.2
Deacon, R.3
York, D.4
Hannan, A.J.5
|