Moyamoya disease factor RNF213 is a giant E3 ligase with a dynein-like core and a distinct ubiquitin-transfer mechanism

Moyamoya disease factor RNF213 is a giant E3 ligase with a dynein-like core and a distinct ubiquitin-transfer mechanism

RNF213 is the major susceptibility factor for Moyamoya disease, a progressive cerebrovascular disorder that often leads to brain stroke in adults and children. Characterization of disease-associated mutations has been complicated by the enormous size of RNF213. Here, we present the cryo-EM structure...

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Bibliographic Details
Main Authors: Juraj Ahel, Anita Lehner, Antonia Vogel, Alexander Schleiffer, Anton Meinhart, David Haselbach, Tim Clausen
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2020-06-01
Series:eLife
Subjects:
protein ubiquitination
protein quality control
molecular machines
cerebrovascular disorder
rare disease
Online Access:https://elifesciences.org/articles/56185

Internet

https://elifesciences.org/articles/56185

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