Structural basis of impaired disaggregase function in the oxidation-sensitive SKD3 mutant causing 3-methylglutaconic aciduria

Abstract Mitochondria are critical to cellular and organismal health. To prevent damage, mitochondria have evolved protein quality control machines to survey and maintain the mitochondrial proteome. SKD3, also known as CLPB, is a ring-forming, ATP-fueled protein disaggregase essential for preserving...

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Bibliographic Details
Main Authors: Sukyeong Lee, Sang Bum Lee, Nuri Sung, Wendy W. Xu, Changsoo Chang, Hyun-Eui Kim, Andre Catic, Francis T. F. Tsai
Format: Article
Language:English
Published: Nature Portfolio 2023-04-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-023-37657-9