Oxidation versus aggregation - how do SOD1 mutants cause ALS?

被引:84
作者
Cleveland, DW
Liu, J
机构
[1] Univ Calif San Diego, Ludwig Inst Canc Res, La Jolla, CA 92093 USA
[2] Univ Calif San Diego, Dept Med, La Jolla, CA 92093 USA
[3] Univ Calif San Diego, Dept Neurosci, La Jolla, CA 92093 USA
关键词
D O I
10.1038/82122
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Multiple models have been proposed for the mechanism by which mutant superoxide dismutase 1 induces motor neuron death and amyotrophic lateral sclerosis. A combination of cell biology and genetics may soon lead to an answer.
引用
收藏
页码:1320 / 1321
页数:2
相关论文
共 11 条
[1]   Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis [J].
Bruening, W ;
Roy, J ;
Giasson, B ;
Figlewicz, DA ;
Mushynski, WE ;
Durham, HD .
JOURNAL OF NEUROCHEMISTRY, 1999, 72 (02) :693-699
[2]   Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1 [J].
Bruijn, LI ;
Houseweart, MK ;
Kato, S ;
Anderson, KL ;
Anderson, SD ;
Ohama, E ;
Reaume, AG ;
Scott, RW ;
Cleveland, DW .
SCIENCE, 1998, 281 (5384) :1851-1854
[3]   From Charcot to SOD1: Mechanisms of selective motor neuron death in ALS [J].
Cleveland, DW .
NEURON, 1999, 24 (03) :515-520
[4]   The copper chaperone for superoxide dismutase [J].
Culotta, VC ;
Klomp, LWJ ;
Strain, J ;
Casareno, RLB ;
Krems, B ;
Gitlin, JD .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1997, 272 (38) :23469-23472
[5]   Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS [J].
Durham, HD ;
Roy, J ;
Dong, L ;
Figlewicz, DA .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 1997, 56 (05) :523-530
[6]   Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase [J].
Estévez, AG ;
Crow, JP ;
Sampson, JB ;
Reiter, C ;
Zhuang, YX ;
Richardson, GJ ;
Tarpey, MM ;
Barbeito, L ;
Beckman, JS .
SCIENCE, 1999, 286 (5449) :2498-2500
[7]   Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis [J].
Johnston, JA ;
Dalton, MJ ;
Gurney, ME ;
Kopito, RR .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (23) :12571-12576
[8]   MUTATIONS IN CU/ZN SUPEROXIDE-DISMUTASE GENE ARE ASSOCIATED WITH FAMILIAL AMYOTROPHIC-LATERAL-SCLEROSIS [J].
ROSEN, DR ;
SIDDIQUE, T ;
PATTERSON, D ;
FIGLEWICZ, DA ;
SAPP, P ;
HENTATI, A ;
DONALDSON, D ;
GOTO, J ;
OREGAN, JP ;
DENG, HX ;
RAHMANI, Z ;
KRIZUS, A ;
MCKENNAYASEK, D ;
CAYABYAB, A ;
GASTON, SM ;
BERGER, R ;
TANZI, RE ;
HALPERIN, JJ ;
HERZFELDT, B ;
VANDENBERGH, R ;
HUNG, WY ;
BIRD, T ;
DENG, G ;
MULDER, DW ;
SMYTH, C ;
LAING, NG ;
SORIANO, E ;
PERICAKVANCE, MA ;
HAINES, J ;
ROULEAU, GA ;
GUSELLA, JS ;
HORVITZ, HR ;
BROWN, RH .
NATURE, 1993, 362 (6415) :59-62
[9]  
SUBRAMANIAM JR, 2000, 30 ANN M SOC NEUR NO
[10]  
WILLIAMSON TL, 2000, SCIENCE, V288, pA399