Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations

Cells have evolved specialized protein disaggregases to reverse toxic protein aggregation and restore protein functionality. In nonmetazoan eukaryotes, the AAA+ disaggregase Hsp78 resolubilizes and reactivates proteins in mitochondria. Curiously, metazoa lack Hsp78. Hence, whether metazoan mitochond...

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Bibliographic Details
Main Authors: Ryan R Cupo, James Shorter
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2020-06-01
Series:eLife
Subjects:
Online Access:https://elifesciences.org/articles/55279