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• W4297373146 endingPage "111408" @default.
• W4297373146 startingPage "111408" @default.
• W4297373146 abstract "The AAA+ protein, Skd3 (human CLPB), solubilizes proteins in the mitochondrial intermembrane space, which is critical for human health. Skd3 variants with defective protein-disaggregase activity cause severe congenital neutropenia (SCN) and 3-methylglutaconic aciduria type 7 (MGCA7). How Skd3 disaggregates proteins remains poorly understood. Here, we report a high-resolution structure of a Skd3-substrate complex. Skd3 adopts a spiral hexameric arrangement that engages substrate via pore-loop interactions in the nucleotide-binding domain (NBD). Substrate-bound Skd3 hexamers stack head-to-head via unique, adaptable ankyrin-repeat domain (ANK)-mediated interactions to form dodecamers. Deleting the ANK linker region reduces dodecamerization and disaggregase activity. We elucidate apomorphic features of the Skd3 NBD and C-terminal domain that regulate disaggregase activity. We also define how Skd3 subunits collaborate to disaggregate proteins. Importantly, SCN-linked subunits sharply inhibit disaggregase activity, whereas MGCA7-linked subunits do not. These advances illuminate Skd3 structure and mechanism, explain SCN and MGCA7 inheritance patterns, and suggest therapeutic strategies." @default.
• W4297373146 created "2022-09-28" @default.
• W4297373146 creator A5000825288 @default.
• W4297373146 creator A5028038243 @default.
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• W4297373146 creator A5050299360 @default.
• W4297373146 creator A5073454413 @default.
• W4297373146 creator A5084836766 @default.
• W4297373146 creator A5090663936 @default.
• W4297373146 date "2022-09-01" @default.
• W4297373146 modified "2023-09-26" @default.
• W4297373146 title "Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3" @default.
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