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Article

HAX1 drives assembly and activation of the mitochondrial intermembrane space chaperone CLPB

2026-05-26

Abstract excerpt

The multicellular metazoan lineage acquired a novel chaperone in the mitochondrial intermembrane space, the AAA+ disaggregase and refoldase CLPB. Although it is not known how they function together, CLPB and the intrinsically disordered IMS protein HAX1 interact and share disease and cellular phenotypes; loss of function in either gene causes severe congenital neutropenia as well as neuropathology and causes many...

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Identifiers and source

Literature Corpus work
66059038-a32d-55ea-be15-d4245b2759ef
DOI
10.64898/2026.05.24.727526
Open publication

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