Article

PEX1 <sup>G843D</sup> remains functional in peroxisome biogenesis but is rapidly degraded by the proteasome

2024-12-13

Abstract excerpt

The PEX1/PEX6 AAA-ATPase is required for the biogenesis and maintenance of peroxisomes. Mutations in HsPEX1 and HsPEX6 disrupt peroxisomal matrix protein import and are the leading cause of Peroxisome Biogenesis Disorders (PBDs). The most common disease-causing mutation in PEX1 is the Hs PEX1 G843D allele, which results in a reduction of peroxisomal protein import. Here we demonstrate that in vitro the homolo...

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Literature Corpus work: 0a3e7f97-9774-5859-a70e-3ba777279e44
DOI: 10.1101/2024.12.10.627778

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