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Title 

cIAPs promote the proteasomal degradation of mutant SOD1 linked to familial amyotrophic lateral sclerosis

 

가족성 근위축성 측삭경화증 연관 돌연변이 SOD1 단백질의 cIAP에 의한 분해

Authors 

Jin Sun ChoiKidae KimD H LeeS ChoJ D HaByoung Chul ParkSunhong KimSung Goo ParkJeong Hoon Kim

Publisher 

Elsevier

Issue Date 

2016

Citation 

Biochemical and Biophysical Research Communications, vol. 480, no. 3, pp. 422-428

Keywords 

cIAPsFALSSOD1Ubiquitination

Abstract 

Although the ubiquitin?proteasome system is believed to play an important role in the pathogenesis of familial amyotrophic lateral sclerosis (FALS), caused by mutations in Cu/Zn-superoxide dismutase 1 (SOD1), the mechanism of how mutant SOD1 protein is regulated in cells is still poorly understood. Here we have demonstrated that cellular inhibitor of apoptosis proteins (cIAPs) are specifically associated with FALS-linked mutant SOD1 (mSOD1) and that this interaction promotes the ubiquitin-dependent proteasomal degradation of mutant SOD1. By utilizing cumate inducible SOD1 cells, we also showed that knock-down or pharmacologic depletion of cIAPs leads to H2O2 induced cytotoxicity in mSOD1 expressing cells. Altogether, our results reveal a novel role of cIAPs in FALS-associated mutant SOD1 regulation.

ISSN 

0006-291X

Link 

http://dx.doi.org/10.1016/j.bbrc.2016.http://dx.doi.org/10.065

Appears in Collections

1. Journal Articles > Journal Articles

Registered Date

2019-05-02


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