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
相关论文
共 38 条
[1]  
BAILEY CK, 1998, AM J HUM GENET S, V63, pA8
[2]   Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 CAG triplet repeat length [J].
Becher, MW ;
Kotzuk, JA ;
Sharp, AH ;
Davies, SW ;
Bates, GP ;
Price, DL ;
Ross, CA .
NEUROBIOLOGY OF DISEASE, 1998, 4 (06) :387-397
[3]  
CHAI Y, IN PRESS HUM MOL GEN
[4]   Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture [J].
Cooper, JK ;
Schilling, G ;
Peters, MF ;
Herring, WJ ;
Sharp, AH ;
Kaminsky, Z ;
Masone, J ;
Khan, FA ;
Delanoy, M ;
Borchelt, DR ;
Dawson, VL ;
Dawson, TM ;
Ross, CA .
HUMAN MOLECULAR GENETICS, 1998, 7 (05) :783-790
[5]   Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 [J].
Cummings, CJ ;
Mancini, MA ;
Antalffy, B ;
DeFranco, DB ;
Orr, HT ;
Zoghbi, HY .
NATURE GENETICS, 1998, 19 (02) :148-154
[6]   Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation [J].
Davies, SW ;
Turmaine, M ;
Cozens, BA ;
DiFiglia, M ;
Sharp, AH ;
Ross, CA ;
Scherzinger, E ;
Wanker, EE ;
Mangiarini, L ;
Bates, GP .
CELL, 1997, 90 (03) :537-548
[7]   Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain [J].
DiFiglia, M ;
Sapp, E ;
Chase, KO ;
Davies, SW ;
Bates, GP ;
Vonsattel, JP ;
Aronin, N .
SCIENCE, 1997, 277 (5334) :1990-1993
[8]   Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract [J].
Goldberg, YP ;
Nicholson, DW ;
Rasper, DM ;
Kalchman, MA ;
Koide, HB ;
Graham, RK ;
Bromm, M ;
KazemiEsfarjani, P ;
Thornberry, NA ;
Vaillancourt, JP ;
Hayden, MR .
NATURE GENETICS, 1996, 13 (04) :442-449
[9]   Genetic classification of primary neurodegenerative disease [J].
Hardy, J ;
Gwinn-Hardy, K .
SCIENCE, 1998, 282 (5391) :1075-1079
[10]   Structure, organization, and dynamics of promyelocytic leukemia protein nuclear bodies [J].
Hodges, M ;
Tissot, C ;
Howe, K ;
Grimwade, D ;
Freemont, PS .
AMERICAN JOURNAL OF HUMAN GENETICS, 1998, 63 (02) :297-304