Protein fate in neurodegenerative proteinopathies: Polyglutamine diseases join the (mis)fold

被引:164
作者
Paulson, HL [1 ]
机构
[1] Univ Iowa, Coll Med, Dept Neurol, Iowa City, IA 52242 USA
关键词
D O I
10.1086/302269
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
[No abstract available]
引用
收藏
页码:339 / 345
页数:7
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共 38 条
[31]   Genetic neurodegenerative diseases: The human illness and transgenic models [J].
Price, DL ;
Sisodia, SS ;
Borchelt, DR .
SCIENCE, 1998, 282 (5391) :1079-1083
[32]   Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA [J].
Reddy, PH ;
Williams, M ;
Charles, V ;
Garrett, L ;
Pike-Buchanan, L ;
Whetsell, WO ;
Miller, G ;
Tagle, DA .
NATURE GENETICS, 1998, 20 (02) :198-202
[33]   Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions [J].
Saudou, F ;
Finkbeiner, S ;
Devys, D ;
Greenberg, ME .
CELL, 1998, 95 (01) :55-66
[34]   Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo [J].
Scherzinger, E ;
Lurz, R ;
Turmaine, M ;
Mangiarini, L ;
Hollenbach, B ;
Hasenbank, R ;
Bates, GP ;
Davies, SW ;
Lehrach, H ;
Wanker, EE .
CELL, 1997, 90 (03) :549-558
[35]   SH3GL3 associates with the huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates [J].
Sittler, A ;
Walter, S ;
Wedemeyer, N ;
Hasenbank, R ;
Scherzinger, E ;
Eickhoff, H ;
Bates, GP ;
Lehrach, H ;
Wanker, EE .
MOLECULAR CELL, 1998, 2 (04) :427-436
[36]   POLYGLUTAMINE EXPANSION AS A PATHOLOGICAL EPITOPE IN HUNTINGTONS-DISEASE AND 4 DOMINANT CEREBELLAR ATAXIAS [J].
TROTTIER, Y ;
LUTZ, Y ;
STEVANIN, G ;
IMBERT, G ;
DEVYS, D ;
CANCEL, G ;
SAUDOU, F ;
WEBER, C ;
DAVID, G ;
TORA, L ;
AGID, Y ;
BRICE, A ;
MANDEL, JL .
NATURE, 1995, 378 (6555) :403-406
[37]   Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila [J].
Warrick, JM ;
Paulson, HL ;
Gray-Board, GL ;
Bui, QT ;
Fischbeck, KH ;
Pittman, RN ;
Bonini, NM .
CELL, 1998, 93 (06) :939-949
[38]   Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract [J].
Wellington, CL ;
Ellerby, LM ;
Hackam, AS ;
Margolis, RL ;
Trifiro, MA ;
Singaraja, R ;
McCutcheon, K ;
Salvesen, GS ;
Propp, SS ;
Bromm, M ;
Rowland, KJ ;
Zhang, TQ ;
Rasper, D ;
Roy, S ;
Thornberry, N ;
Pinsky, L ;
Kakizuka, A ;
Ross, CA ;
Nicholson, DW ;
Bredesen, DE ;
Hayden, MR .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1998, 273 (15) :9158-9167