Article

ASPL-driven subunit exchange remodels VCP/p97 hexamers and is impaired by a multisystem proteinopathy mutation

2026-05-08

Abstract excerpt

Valosin-containing protein (VCP/p97) is an essential homohexameric AAA+ ATPase that powers ubiquitin-dependent protein quality control by extraction and unfolding of clients for proteasomal degradation. Heterozygous, autosomal-dominant VCP missense mutants are associated with multisystem proteinopathy (MSP) through unclear molecular mechanisms. We developed a single-molecule pull-down assay to quantify VCP hexamer...

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Literature Corpus work: aab5d6ca-4562-5297-8d7a-9a54f6340135
DOI: 10.64898/2026.05.07.723621

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