cIAPs promote the proteasomal degradation of mutant SOD1 linked to familial amyotrophic lateral sclerosis = 가족성 근위축성 측삭경화증 연관 돌연변이 SOD1 단백질의 cIAP에 의한 분해

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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
Author(s)
Jin Sun Choi; Kidae Kim; D H Lee; S Cho; J D Ha; Byoung Chul Park; Sunhong Kim; Sung Goo ParkJeong Hoon Kim
Bibliographic Citation
Biochemical and Biophysical Research Communications, vol. 480, no. 3, pp. 422-428
Publication Year
2016
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.
Keyword
cIAPsFALSSOD1Ubiquitination
ISSN
0006-291X
Publisher
Elsevier
DOI
http://dx.doi.org/10.1016/j.bbrc.2016.10.065
Type
Article
Appears in Collections:
Division of Biomedical Research > Disease Target Structure Research Center > 1. Journal Articles
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