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Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity
被引:157
作者:

Niwa, J
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Ishigaki, S
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Hishikawa, N
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Yamamoto, M
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Doyu, M
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Murata, S
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Tanaka, K
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Taniguchi, N
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan

Sobue, G
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机构: Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan
机构:
[1] Nagoya Univ, Grad Sch Med, Dept Neurol, Showa Ku, Nagoya, Aichi 4668550, Japan
[2] Tokyo Metropolitan Inst Med Sci, Dept Mol Oncol, Bunkyo Ku, Tokyo 1138613, Japan
[3] Osaka Univ, Grad Sch Med, Dept Biochem, Suita, Osaka 5650871, Japan
关键词:
D O I:
10.1074/jbc.M206559200
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:
Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder resulting from the degeneration of motor neurons in the cerebral cortex, brainstem, and spinal cord. The cytopathological hallmark in the remaining motor neurons of ALS is the presence of ubiquitylated inclusions consisting of insoluble protein aggregates. In this paper we report that Dorfin, a RING finger-type E3 ubiquitin ligase, is predominantly localized in the inclusion bodies of familial ALS with a copper/zinc superoxide dismutase (SOD1) mutation as well as sporadic AILS. Dorfin physically bound and ubiquitylated various SOD1 mutants derived from familial AILS patients and enhanced their degradation, but it had no effect on the stability of the wild-type SOD1. The overexpression of Dorfin protected against the toxic effects of mutant SOD1 on neural cells and reduced SOD1 inclusions. Our results indicate that Dorfin protects neurons by recognizing and then ubiquitylating mutant SOD1 proteins followed by targeting them for proteasomal degradation.
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页码:36793 / 36798
页数:6
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