Article

Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3

2022-02-17

Abstract excerpt

<h4>Summary</h4> The AAA+ protein, Skd3 (human CLPB ), solubilizes proteins in the mitochondrial intermembrane space, which is critical for human health. Skd3 variants with impaired protein-disaggregase activity cause severe congenital neutropenia (SCN) and 3-methylglutaconic aciduria type 7 (MGCA7). Yet how Skd3 disaggregates proteins remains poorly understood. Here, we report a high-resolution structure of a S...

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Literature Corpus work: 73709b77-3dbf-559e-a1be-ab223a7f7f98
DOI: 10.1101/2022.02.17.480866

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