Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity

Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity

The ubiquitin–proteasome system (UPS) is involved in the pathogenetic mechanisms of neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). Dorfin is a ubiquitin ligase (E3) that degrades mutant SOD1 proteins, which are responsible for familial ALS. Although Dorfin has potential...

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Bibliographic Details
Main Authors: Shinsuke Ishigaki, Jun-ichi Niwa, Shin-ichi Yamada, Miho Takahashi, Takashi Ito, Jun Sone, Manabu Doyu, Fumihiko Urano, Gen Sobue
Format: Article
Language:English
Published: Elsevier 2007-02-01
Series:Neurobiology of Disease
Subjects:
Dorfin
ALS
SOD1
CHIP
Neurodegeneration
Ubiquitin–proteasome system
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996106002440

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