-
3
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
-
The Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72:971-983, 1993.
-
(1993)
Cell
, vol.72
, pp. 971-983
-
-
-
4
-
-
0344961865
-
The use of transgenic and knock-in mice to study Huntington's disease
-
Hickey MA, Chesselet MF. The use of transgenic and knock-in mice to study Huntington's disease. Cytogenet Genome Res 100: 276-286, 2003.
-
(2003)
Cytogenet Genome Res
, vol.100
, pp. 276-286
-
-
Hickey, M.A.1
Chesselet, M.F.2
-
5
-
-
0029082383
-
Inactivation of the mouse Huntington's disease gene homolog Hdh
-
Duyao MP, Auerbach AB, Ryan A, Persichetti F, Barnes GT, McNeil SM, et al. Inactivation of the mouse Huntington's disease gene homolog Hdh. Science 269:407-410, 1995.
-
(1995)
Science
, vol.269
, pp. 407-410
-
-
Duyao, M.P.1
Auerbach, A.B.2
Ryan, A.3
Persichetti, F.4
Barnes, G.T.5
McNeil, S.M.6
-
6
-
-
0029055717
-
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
-
Nasir J, Floresco SB, O'Kusky JR, Diewert VM, Richman JM, Zeisler J, et al. Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81:811-823, 1995.
-
(1995)
Cell
, vol.81
, pp. 811-823
-
-
Nasir, J.1
Floresco, S.B.2
O'Kusky, J.R.3
Diewert, V.M.4
Richman, J.M.5
Zeisler, J.6
-
7
-
-
84993912315
-
Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
-
Zeitlin S, Liu JP, Chapman DL, Papaioannou VE, Efstratiadis A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nat Genet 11:155-163, 1995.
-
(1995)
Nat Genet
, vol.11
, pp. 155-163
-
-
Zeitlin, S.1
Liu, J.P.2
Chapman, D.L.3
Papaioannou, V.E.4
Efstratiadis, A.5
-
8
-
-
0030613177
-
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
-
White JK, Auerbach W, Duyao MP, Vonsattel JP, Gusella JF, Joyner AL, et al. Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nat Genet 17:404-410, 1997.
-
(1997)
Nat Genet
, vol.17
, pp. 404-410
-
-
White, J.K.1
Auerbach, W.2
Duyao, M.P.3
Vonsattel, J.P.4
Gusella, J.F.5
Joyner, A.L.6
-
9
-
-
0033757718
-
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
-
Dragatsis I, Levine MS, Zeitlin S. Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet 26:300-306, 2000.
-
(2000)
Nat Genet
, vol.26
, pp. 300-306
-
-
Dragatsis, I.1
Levine, M.S.2
Zeitlin, S.3
-
10
-
-
0032949459
-
A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
-
Shelbourne PF, Killeen N, Hevner RF, Johnston HM, Tecott L, Lewandoski M, et al. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Hum Mol Genet 8:763-774, 1999.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 763-774
-
-
Shelbourne, P.F.1
Killeen, N.2
Hevner, R.F.3
Johnston, H.M.4
Tecott, L.5
Lewandoski, M.6
-
11
-
-
18544410106
-
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90:537-548, 1997.
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
DiFiglia, M.4
Sharp, A.H.5
Ross, C.A.6
-
12
-
-
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 K, Seller M, Cozens B, Harper A, Hetherington C, 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 87:493-506, 1996.
-
(1996)
Cell
, vol.87
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
Cozens, B.4
Harper, A.5
Hetherington, C.6
-
14
-
-
0041691176
-
Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats
-
Menalled LB, Sison JD, Dragatsis I, Zeitlin S, Chesselet MF. 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 465:11-26, 2003.
-
(2003)
J Comp Neurol
, vol.465
, pp. 11-26
-
-
Menalled, L.B.1
Sison, J.D.2
Dragatsis, I.3
Zeitlin, S.4
Chesselet, M.F.5
-
15
-
-
0037107191
-
Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice
-
Menalled LB, Sison JD, Wu Y, Olivieri M, Li XJ, Li H, et al. Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. J Neurosci 22: 8266-8276, 2002.
-
(2002)
J Neurosci
, vol.22
, pp. 8266-8276
-
-
Menalled, L.B.1
Sison, J.D.2
Wu, Y.3
Olivieri, M.4
Li, X.J.5
Li, H.6
-
16
-
-
0034329159
-
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
-
Gusella JF, MacDonald ME. Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat Rev Neurosci 1:109-115, 2000.
-
(2000)
Nat Rev Neurosci
, vol.1
, pp. 109-115
-
-
Gusella, J.F.1
MacDonald, M.E.2
-
17
-
-
0034598808
-
Motor disorder in Huntington's disease begins as a dysfunction in error feedback control
-
Smith MA, Brandt J, Shadmehr R. Motor disorder in Huntington's disease begins as a dysfunction in error feedback control. Nature 403:544-549, 2000.
-
(2000)
Nature
, vol.403
, pp. 544-549
-
-
Smith, M.A.1
Brandt, J.2
Shadmehr, R.3
-
18
-
-
0037087771
-
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
-
Wheeler VC, Gutekunst CA, Vrbanac V, Lebel LA, Schilling G, Hersch S, et al. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet 11:633-640, 2002.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 633-640
-
-
Wheeler, V.C.1
Gutekunst, C.A.2
Vrbanac, V.3
Lebel, L.A.4
Schilling, G.5
Hersch, S.6
-
19
-
-
0035862896
-
Neurological abnormalities in a knock-in mouse model of Huntington's disease
-
Lin CH, Tallaksen-Greene S, Chien WM, Cearley JA, Jackson WS, Grouse AB, et al. Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet 10:137-144, 2001.
-
(2001)
Hum Mol Genet
, vol.10
, pp. 137-144
-
-
Lin, C.H.1
Tallaksen-Greene, S.2
Chien, W.M.3
Cearley, J.A.4
Jackson, W.S.5
Grouse, A.B.6
-
20
-
-
0033571743
-
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
-
Levine MS, Klapstein GJ, Koppel A, Gruen E, Cepeda C, Vargas ME, et al. Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease. J Neurosci Res 58:515-532, 1999.
-
(1999)
J Neurosci Res
, vol.58
, pp. 515-532
-
-
Levine, M.S.1
Klapstein, G.J.2
Koppel, A.3
Gruen, E.4
Cepeda, C.5
Vargas, M.E.6
-
21
-
-
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, Vrbanac V, Weaver M, Li XJ, 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 9:503-513, 2000.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 503-513
-
-
Wheeler, V.C.1
White, J.K.2
Gutekunst, C.A.3
Vrbanac, V.4
Weaver, M.5
Li, X.J.6
-
22
-
-
0000790724
-
SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes
-
Liu YF, Deth RC, Devys D. SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J Biol Chem 272:8121-8124, 1997.
-
(1997)
J Biol Chem
, vol.272
, pp. 8121-8124
-
-
Liu, Y.F.1
Deth, R.C.2
Devys, D.3
-
23
-
-
0034284565
-
Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis
-
Passani LA, Bedford MT, Faber PW, McGinnis KM, Sharp AH, Gusella JF, et al. Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis. Hum Mol Genet 9:2175-2182, 2000.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 2175-2182
-
-
Passani, L.A.1
Bedford, M.T.2
Faber, P.W.3
McGinnis, K.M.4
Sharp, A.H.5
Gusella, J.F.6
-
24
-
-
0031662269
-
Huntingtin interacts with a family of WW domain proteins
-
Faber PW, Barnes GT, Srinidhi J, Chen J, Gusella JF, MacDonald ME. Huntingtin interacts with a family of WW domain proteins. Hum Mol Genet 7:1463-1474, 1998.
-
(1998)
Hum Mol Genet
, vol.7
, pp. 1463-1474
-
-
Faber, P.W.1
Barnes, G.T.2
Srinidhi, J.3
Chen, J.4
Gusella, J.F.5
MacDonald, M.E.6
-
25
-
-
0032186107
-
SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates
-
Sittler A, Walter S, Wedemeyer N, Hasenbank R, Scherzinger E, Eickhoff H, et al. SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell 2:427-436, 1998.
-
(1998)
Mol Cell
, vol.2
, pp. 427-436
-
-
Sittler, A.1
Walter, S.2
Wedemeyer, N.3
Hasenbank, R.4
Scherzinger, E.5
Eickhoff, H.6
-
26
-
-
0842322740
-
Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction
-
Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, et al. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. J Neurosci 24:269-281, 2004.
-
(2004)
J Neurosci
, vol.24
, pp. 269-281
-
-
Qin, Z.H.1
Wang, Y.2
Sapp, E.3
Cuiffo, B.4
Wanker, E.5
Hayden, M.R.6
-
27
-
-
0032938295
-
Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
-
Wheeler VC, Auerbach W, White JK, Srinidhi J, Auerbach A, Ryan A, et al. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 8:115-122, 1999.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 115-122
-
-
Wheeler, V.C.1
Auerbach, W.2
White, J.K.3
Srinidhi, J.4
Auerbach, A.5
Ryan, A.6
-
28
-
-
0032897760
-
Impaired synaptic plasticity in mice carrying the Huntington's disease mutation
-
Usdin MT, Shelbourne PF, Myers RM, Madison DV. Impaired synaptic plasticity in mice carrying the Huntington's disease mutation. Hum Mol Genet 8:839-846, 1999.
-
(1999)
Hum Mol Genet
, vol.8
, pp. 839-846
-
-
Usdin, M.T.1
Shelbourne, P.F.2
Myers, R.M.3
Madison, D.V.4
-
29
-
-
17344363559
-
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
-
Martindale D, Hackam A, Wieczorek A, Ellerby L, Wellington C, McCutcheon K, et al. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet 18:150-154, 1998.
-
(1998)
Nat Genet
, vol.18
, pp. 150-154
-
-
Martindale, D.1
Hackam, A.2
Wieczorek, A.3
Ellerby, L.4
Wellington, C.5
McCutcheon, K.6
-
30
-
-
9344227302
-
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
-
Goldberg YP, Nicholson DW, Rasper DM, Kalchman MA, Koide HB, Graham RK, et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat Genet 13:442-449, 1996.
-
(1996)
Nat Genet
, vol.13
, pp. 442-449
-
-
Goldberg, Y.P.1
Nicholson, D.W.2
Rasper, D.M.3
Kalchman, M.A.4
Koide, H.B.5
Graham, R.K.6
-
31
-
-
0035969336
-
Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: Behavioral testing and impact of diabetes mellitus
-
Luesse HG, Schiefer J, Spruenken A, Puls C, Block F, Kosinski CM. Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: behavioral testing and impact of diabetes mellitus. Behav Brain Res 126:185-195, 2001.
-
(2001)
Behav Brain Res
, vol.126
, pp. 185-195
-
-
Luesse, H.G.1
Schiefer, J.2
Spruenken, A.3
Puls, C.4
Block, F.5
Kosinski, C.M.6
-
32
-
-
0033614772
-
Transgenic mice expressing mutated full-length HD cDNA: A paradigm for locomotor changes and selective neuronal loss in Huntington's disease
-
Reddy PH, Charles V, Williams M, Miller G, Whetsell WO Jr, Tagle DA. Transgenic mice expressing mutated full-length HD cDNA: a paradigm for locomotor changes and selective neuronal loss in Huntington's disease. Philos Trans R Soc Lond B Biol Sci 354:1035-1045, 1999.
-
(1999)
Philos Trans R Soc Lond B Biol Sci
, vol.354
, pp. 1035-1045
-
-
Reddy, P.H.1
Charles, V.2
Williams, M.3
Miller, G.4
Whetsell Jr., W.O.5
Tagle, D.A.6
-
33
-
-
0033560924
-
Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
-
Carter RJ, Lione LA, Humby T, Mangiarini L, Mahal A, Bates GP, et al. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 19:3248-3257, 1999.
-
(1999)
J Neurosci
, vol.19
, pp. 3248-3257
-
-
Carter, R.J.1
Lione, L.A.2
Humby, T.3
Mangiarini, L.4
Mahal, A.5
Bates, G.P.6
-
34
-
-
10744227174
-
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
-
Slow EJ, van Raamsdonk J, Rogers D, Coleman SH, Graham RK, Deng Y, et al. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 12:1555-1567, 2003.
-
(2003)
Hum Mol Genet
, vol.12
, pp. 1555-1567
-
-
Slow, E.J.1
Van Raamsdonk, J.2
Rogers, D.3
Coleman, S.H.4
Graham, R.K.5
Deng, Y.6
-
35
-
-
0037444445
-
Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease
-
Yu ZX, Li SH, Evans J, Pillarisetti A, Li H, Li XJ. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci 23:2193-2202, 2003.
-
(2003)
J Neurosci
, vol.23
, pp. 2193-2202
-
-
Yu, Z.X.1
Li, S.H.2
Evans, J.3
Pillarisetti, A.4
Li, H.5
Li, X.J.6
-
36
-
-
0035503511
-
Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice
-
Li H, Li SH, Yu ZX, Shelbourne P, Li XJ. Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice. J Neurosci 21:8473-8481, 2001.
-
(2001)
J Neurosci
, vol.21
, pp. 8473-8481
-
-
Li, H.1
Li, S.H.2
Yu, Z.X.3
Shelbourne, P.4
Li, X.J.5
-
37
-
-
0002885477
-
Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations
-
(Chase TN, Wexler NS, Barbeau A, eds). New York: Raven Press
-
Roizin L, Stellar S, Liu JC. Neuronal nuclear-cytoplasmic changes in Huntington's chorea: electron microscope investigations. In: Advances in neurology (Chase TN, Wexler NS, Barbeau A, eds), pp 95-122. New York: Raven Press, 1979.
-
(1979)
Advances in Neurology
, pp. 95-122
-
-
Roizin, L.1
Stellar, S.2
Liu, J.C.3
-
38
-
-
0028799793
-
Early loss of neostriatal striosome neurons in Huntington's disease
-
Hedreen JC, Folstein SE. Early loss of neostriatal striosome neurons in Huntington's disease. J Neuropathol Exp Neurol 54:105-120, 1995.
-
(1995)
J Neuropathol Exp Neurol
, vol.54
, pp. 105-120
-
-
Hedreen, J.C.1
Folstein, S.E.2
-
39
-
-
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 95:55-66, 1998.
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
40
-
-
0037154229
-
Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment
-
Muchowski PJ, Ning K, D'Souza-Schorey C, Fields S. Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. Proc Natl Acad Sci USA 99:727-732, 2002.
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 727-732
-
-
Muchowski, P.J.1
Ning, K.2
D'Souza-Schorey, C.3
Fields, S.4
-
41
-
-
7244236320
-
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
-
Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431:805-810, 2004.
-
(2004)
Nature
, vol.431
, pp. 805-810
-
-
Arrasate, M.1
Mitra, S.2
Schweitzer, E.S.3
Segal, M.R.4
Finkbeiner, S.5
-
42
-
-
0032987513
-
Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease
-
Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, DiFiglia M. Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. J Neuropathol Exp Neurol 58:165-173, 1999.
-
(1999)
J Neuropathol Exp Neurol
, vol.58
, pp. 165-173
-
-
Sapp, E.1
Penney, J.2
Young, A.3
Aronin, N.4
Vonsattel, J.P.5
DiFiglia, M.6
-
43
-
-
0036453663
-
Evidence for dysfunction of the nigrostriatal pathway in the R6/1 line of transgenic Huntington's disease mice
-
Petersen A, Puschban Z, Lotharius J, NicNiocaill B, Wiekop P, O'Connor WT, et al. Evidence for dysfunction of the nigrostriatal pathway in the R6/1 line of transgenic Huntington's disease mice. Neurobiol Dis 11:134-146, 2002.
-
(2002)
Neurobiol Dis
, vol.11
, pp. 134-146
-
-
Petersen, A.1
Puschban, Z.2
Lotharius, J.3
Nicniocaill, B.4
Wiekop, P.5
O'Connor, W.T.6
-
44
-
-
0035889973
-
Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease
-
Martin-Aparicio E, Yamamoto A, Hernandez F, Hen R, Avila J, Lucas JJ. Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease. J Neurosci 21:8772-8781, 2001.
-
(2001)
J Neurosci
, vol.21
, pp. 8772-8781
-
-
Martin-Aparicio, E.1
Yamamoto, A.2
Hernandez, F.3
Hen, R.4
Avila, J.5
Lucas, J.J.6
-
45
-
-
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 LM, Toneff T, Hwang SR, Chesselet MF, Hook VY. 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 21:1830-1837, 2001.
-
(2001)
J Neurosci
, vol.21
, pp. 1830-1837
-
-
Mende-Mueller, L.M.1
Toneff, T.2
Hwang, S.R.3
Chesselet, M.F.4
Hook, V.Y.5
-
46
-
-
2442631459
-
Inhibition of calpain cleavage of huntingtin reduces toxicity: Accumulation of calpain/caspase fragments in the nucleus
-
Gafni J, Hermel E, Young JE, Wellington CL, Hayden MR, Ellerby LM. Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. J Biol Chem 279:20211-20220, 2004.
-
(2004)
J Biol Chem
, vol.279
, pp. 20211-20220
-
-
Gafni, J.1
Hermel, E.2
Young, J.E.3
Wellington, C.L.4
Hayden, M.R.5
Ellerby, L.M.6
-
47
-
-
0034641887
-
Dramatic mutation instability in HD mouse striatum: Does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?
-
Kennedy L, Shelbourne PF. Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease? Hum Mol Genet 9:2539-2544, 2000.
-
(2000)
Hum Mol Genet
, vol.9
, pp. 2539-2544
-
-
Kennedy, L.1
Shelbourne, P.F.2
-
48
-
-
0346752132
-
Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis
-
Kennedy L, Evans E, Chen CM, Craven L, Detloff PJ, Ennis M, et al. Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis. Hum Mol Genet 12:3359-3367, 2003.
-
(2003)
Hum Mol Genet
, vol.12
, pp. 3359-3367
-
-
Kennedy, L.1
Evans, E.2
Chen, C.M.3
Craven, L.4
Detloff, P.J.5
Ennis, M.6
-
49
-
-
0037321290
-
Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum
-
Wheeler VC, Lebel LA, Vrbanac V, Teed A, te Riele H, MacDonald ME. Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum. Hum Mol Genet 12:273-281, 2003.
-
(2003)
Hum Mol Genet
, vol.12
, pp. 273-281
-
-
Wheeler, V.C.1
Lebel, L.A.2
Vrbanac, V.3
Teed, A.4
Te Riele, H.5
MacDonald, M.E.6
-
50
-
-
0034426013
-
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
-
Li H, Li SH, Johnston H, Shelbourne PF, Li XJ. Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nat Genet 25:385-389, 2000.
-
(2000)
Nat Genet
, vol.25
, pp. 385-389
-
-
Li, H.1
Li, S.H.2
Johnston, H.3
Shelbourne, P.F.4
Li, X.J.5
-
51
-
-
0001589776
-
Differential loss of striatal projection neurons in Huntington disease
-
Reiner A, Albin RL, Anderson KD, D'Amato CJ, Penney JB, Young AB. Differential loss of striatal projection neurons in Huntington disease. Proc Natl Acad Sci USA 85:5733-5737, 1988.
-
(1988)
Proc Natl Acad Sci USA
, vol.85
, pp. 5733-5737
-
-
Reiner, A.1
Albin, R.L.2
Anderson, K.D.3
D'Amato, C.J.4
Penney, J.B.5
Young, A.B.6
-
52
-
-
2942625910
-
Differential loss of striatal projection systems in Huntington's disease: A quantitative immunohistochemical study
-
Deng YP, Albin RL, Penney JB, Young AB, Anderson KD, Reiner A. Differential loss of striatal projection systems in Huntington's disease: a quantitative immunohistochemical study. J Chem Neuroanat 27:143-164, 2004.
-
(2004)
J Chem Neuroanat
, vol.27
, pp. 143-164
-
-
Deng, Y.P.1
Albin, R.L.2
Penney, J.B.3
Young, A.B.4
Anderson, K.D.5
Reiner, A.6
-
53
-
-
0028873186
-
Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis
-
Sapp E, Ge P, Aizawa H, Bird E, Penney J, Young AB, et al. Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis. Neuroscience 64:397-404, 1995.
-
(1995)
Neuroscience
, vol.64
, pp. 397-404
-
-
Sapp, E.1
Ge, P.2
Aizawa, H.3
Bird, E.4
Penney, J.5
Young, A.B.6
-
54
-
-
1542267796
-
Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease
-
Lee WC, Yoshihara M, Littleton JT. Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc Natl Acad Sci USA 101:3224-3229, 2004.
-
(2004)
Proc Natl Acad Sci USA
, vol.101
, pp. 3224-3229
-
-
Lee, W.C.1
Yoshihara, M.2
Littleton, J.T.3
-
55
-
-
0028803757
-
A huntingtin-associated protein enriched in brain with implications for pathology
-
Li XJ, Li SH, Sharp AH, Nucifora FC Jr, Schilling G, Lanahan A, et al. A huntingtin-associated protein enriched in brain with implications for pathology. Nature 378:398-402, 1995.
-
(1995)
Nature
, vol.378
, pp. 398-402
-
-
Li, X.J.1
Li, S.H.2
Sharp, A.H.3
Nucifora Jr., F.C.4
Schilling, G.5
Lanahan, A.6
-
56
-
-
0032519646
-
Interaction of huntingtin-associated protein with dynactin P150Glued
-
Li SH, Gutekunst CA, Hersch SM, Li XJ. Interaction of huntingtin-associated protein with dynactin P150Glued. J Neurosci 18:1261-1269, 1998.
-
(1998)
J Neurosci
, vol.18
, pp. 1261-1269
-
-
Li, S.H.1
Gutekunst, C.A.2
Hersch, S.M.3
Li, X.J.4
-
57
-
-
0030726894
-
Huntingtin-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin
-
Engelender S, Sharp AH, Colomer V, Tokito MK, Lanahan A, Worley P, et al. Huntingtin-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin. Hum Mol Genet 6:2205-2212, 1997.
-
(1997)
Hum Mol Genet
, vol.6
, pp. 2205-2212
-
-
Engelender, S.1
Sharp, A.H.2
Colomer, V.3
Tokito, M.K.4
Lanahan, A.5
Worley, P.6
-
58
-
-
10744224530
-
Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport
-
Szebenyi G, Morfini GA, Babcock A, Gould M, Selkoe K, Stenoien DL, et al. Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport. Neuron 40:41-52, 2003.
-
(2003)
Neuron
, vol.40
, pp. 41-52
-
-
Szebenyi, G.1
Morfini, G.A.2
Babcock, A.3
Gould, M.4
Selkoe, K.5
Stenoien, D.L.6
-
59
-
-
85030740919
-
Genetic background modifies early disease events in the striatum of Huntington's disease knock-in mice
-
Paper presented Toronto, Ontario, Canada (Abstract 2203)
-
Lloret A, Janice E, Teed A, Hakky M, MacDonald ME, Wheeler VC. Genetic background modifies early disease events in the striatum of Huntington's disease knock-in mice. Paper presented at the 54th Annual Meeting of the American Society of Human Genetics, Toronto, Ontario, Canada (Abstract 2203), 2004.
-
(2004)
54th Annual Meeting of the American Society of Human Genetics
-
-
Lloret, A.1
Janice, E.2
Teed, A.3
Hakky, M.4
MacDonald, M.E.5
Wheeler, V.C.6
-
60
-
-
0034283877
-
Transcriptional dysregulation in Huntington's disease
-
Cha JH. Transcriptional dysregulation in Huntington's disease. Trends Neurosci 23:387-392, 2000.
-
(2000)
Trends Neurosci
, vol.23
, pp. 387-392
-
-
Cha, J.H.1
-
61
-
-
0034048845
-
Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease
-
Menalled L, Zanjani H, MacKenzie L, Koppel A, Carpenter E, Zeitlin S, et al. Decrease in striatal enkephalin mRNA in mouse models of Huntington's disease. Exp Neurol 162:328-342, 2000.
-
(2000)
Exp Neurol
, vol.162
, pp. 328-342
-
-
Menalled, L.1
Zanjani, H.2
MacKenzie, L.3
Koppel, A.4
Carpenter, E.5
Zeitlin, S.6
-
62
-
-
0037106316
-
Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice
-
Fossale E, Wheeler VC, Vrbanac V, Lebel LA, Teed A, Mysore JS, et al. Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice. Hum Mol Genet 11:2233-2241, 2002.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2233-2241
-
-
Fossale, E.1
Wheeler, V.C.2
Vrbanac, V.3
Lebel, L.A.4
Teed, A.5
Mysore, J.S.6
-
63
-
-
3042717240
-
Cellular toxicity of polyglutamine expansion proteins: Mechanism of transcription factor deactivation
-
Schaffar G, Breuer P, Boteva R, Behrends C, Tzvetkov N, Strippel N, et al. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell 15:95-105, 2004.
-
(2004)
Mol Cell
, vol.15
, pp. 95-105
-
-
Schaffar, G.1
Breuer, P.2
Boteva, R.3
Behrends, C.4
Tzvetkov, N.5
Strippel, N.6
-
64
-
-
12744279625
-
Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: Neuronal selectivity and potential neuroprotective role of HAP1
-
Zucker B, Luthi-Carter R, Kama JA, Dunah AW, Stern EA, Fox JH, et al. Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1. Hum Mol Genet 14:179-189, 2005.
-
(2005)
Hum Mol Genet
, vol.14
, pp. 179-189
-
-
Zucker, B.1
Luthi-Carter, R.2
Kama, J.A.3
Dunah, A.W.4
Stern, E.A.5
Fox, J.H.6
-
65
-
-
0035919701
-
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
-
Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L, et al. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293:493-498, 2001.
-
(2001)
Science
, vol.293
, pp. 493-498
-
-
Zuccato, C.1
Ciammola, A.2
Rigamonti, D.3
Leavitt, B.R.4
Goffredo, D.5
Conti, L.6
-
66
-
-
3142636768
-
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
-
Gauthier LR, Charrin BC, Borrell-Pages M, Dompierre JP, Rangone H, Cordelieres FP, et al. Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell 118:127-138, 2004.
-
(2004)
Cell
, vol.118
, pp. 127-138
-
-
Gauthier, L.R.1
Charrin, B.C.2
Borrell-Pages, M.3
Dompierre, J.P.4
Rangone, H.5
Cordelieres, F.P.6
-
67
-
-
0034595722
-
Brain-derived neurotrophic factor in Huntington disease
-
Ferrer I, Goutan E, Marin C, Rey MJ, Ribalta T. Brain-derived neurotrophic factor in Huntington disease. Brain Res 866:257-261, 2000.
-
(2000)
Brain Res
, vol.866
, pp. 257-261
-
-
Ferrer, I.1
Goutan, E.2
Marin, C.3
Rey, M.J.4
Ribalta, T.5
-
68
-
-
0037335074
-
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice
-
Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet 12:497-508, 2003.
-
(2003)
Hum Mol Genet
, vol.12
, pp. 497-508
-
-
Gines, S.1
Seong, I.S.2
Fossale, E.3
Ivanova, E.4
Trettel, F.5
Gusella, J.F.6
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