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Article

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

Nature communications - 11 Apr 2023

Lee Sukyeong, Lee Sang Bum, Sung Nuri, Xu Wendy W, Chang Changsoo, Kim Hyun-Eui, Catic Andre, Tsai Francis T F

Abstract excerpt

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 mitochondrial integrity and structure. SKD3 deficiency causes 3-methylglutaconic aciduria type VII (MGCA7) and early death...

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Identifiers

PMID: 37041140
DOI: 10.1038/s41467-023-37657-9
PMCID: PMC10090083

Genes

CLPB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Structural basis of impaired disaggregase function in the oxidation-sensitive SKD3 mutant causing 3-methylglutaconic aciduria.PMID 37041140Read ↗

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