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Title: Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation
Author: Ying, Zheng ; Wang, Hongfeng ; Fan, Huadong ; Zhu, Xiaodong ; null(周嘉伟) ; Fei, Erkang ; Wang, Guanghui
Source: HUMAN MOLECULAR GENETICS
Issued Date: 2009
Volume: 18, Issue:22, Pages:4268-4281
Keyword: AMYOTROPHIC-LATERAL-SCLEROSIS ; RETICULUM-ASSOCIATED DEGRADATION ; MOTOR-NEURON DEATH ; TRANSMEMBRANE CONDUCTANCE REGULATOR ; PROTEIN-QUALITY CONTROL ; MACHADO-JOSEPH-DISEASE ; CELL-RECEPTOR SUBUNITS ; N-TERMINAL HUNTINGTIN ; LINKED MUTANT SOD1 ; HMG-COA REDUCTASE
Subject: Biochemistry & Molecular Biology ; Genetics & Heredity
Corresponding Author: Wang, GH (reprint author), Univ Sci & Technol China, Lab Mol Neuropathol, Hefei Natl Lab Phys Sci Microscale, Hefei 230027, Anhui, Peoples R China,wghui@ustc.edu.cn
English Abstract: Superoxide dismutase-1 (SOD1) and ataxin-3 are two neurodegenerative disease proteins in association with familial amyotrophic lateral sclerosis and Machado-Joseph disease/spinocerebellar ataxia type 3. Both normal and mutant types of SOD1 and ataxin-3 are degraded by the proteasome. It was recently reported that these two proteins are associated with the endoplasmic reticulum (ER). Mammalian gp78 is an E3 ubiquitin ligase involved in ER-associated degradation (ERAD). Here, we show that gp78 interacts with both SOD1 and ataxin-3. Overexpression of gp78 promotes the ubiquitination and degradation of these two proteins, whereas knockdown of gp78 stabilizes them. Moreover, gp78 represses aggregate formation of mutant SOD1 and protect cells against mutant SOD1-induced cell death. Furthermore, gp78 is increased in cells transfected with these two mutant proteins as well as in ALS mice. Thus, our results suggest that gp78 functions in the regulation of SOD1 and ataxin-3 to target them for ERAD.
Indexed Type: sci
Language: 英语
Content Type: 期刊论文
URI: http://ir.sibs.ac.cn/handle/331001/1628
Appears in Collections:神经所(总)_期刊论文
基底神经节的发育与退行性疾病研究组_期刊论文

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Ying, Zheng; Wang, Hongfeng; Fan, Huadong; Zhu, Xiaodong; Zhou, Jiawei; Fei, Erkang; Wang, Guanghui.Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation,HUMAN MOLECULAR GENETICS,2009,18(22):4268-4281
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