Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7

Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7

Abstract Nuclear and mitochondrial genome mutations lead to various mitochondrial diseases, many of which affect the mitochondrial respiratory chain. The proteome of the intermembrane space (IMS) of mitochondria consists of several important assembly factors that participate in the biogenesis of mit...

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Main Authors: Karthik Mohanraj, Michal Wasilewski, Cristiane Benincá, Dominik Cysewski, Jaroslaw Poznanski, Paulina Sakowska, Zaneta Bugajska, Markus Deckers, Sven Dennerlein, Erika Fernandez‐Vizarra, Peter Rehling, Michal Dadlez, Massimo Zeviani, Agnieszka Chacinska
Format: Article
Language:English
Published: Springer Nature 2019-03-01
Series:EMBO Molecular Medicine
Subjects:
COA7/RESA1
mitochondrial disease
proteasome
protein degradation
protein import
Online Access:https://doi.org/10.15252/emmm.201809561
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https://doi.org/10.15252/emmm.201809561

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