Article

Skd3 (human <i>CLPB</i> ) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations

2020-01-18

Abstract excerpt

<h4>ABSTRACT</h4> 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 mitochondria reactivate aggregated proteins is unknown. Here, we establish that a mitochondrial AAA+ protei...

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Literature Corpus work: 890bc2b7-cc82-5efe-b425-c47747e10265
DOI: 10.1101/2020.01.17.911016

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