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  • Title: Role of AAA(+)-proteins in peroxisome biogenesis and function.
    Author: Grimm I, Erdmann R, Girzalsky W.
    Journal: Biochim Biophys Acta; 2016 May; 1863(5):828-37. PubMed ID: 26453804.
    Abstract:
    Mutations in the PEX1 gene, which encodes a protein required for peroxisome biogenesis, are the most common cause of the Zellweger spectrum diseases. The recognition that Pex1p shares a conserved ATP-binding domain with p97 and NSF led to the discovery of the extended family of AAA+-type ATPases. So far, four AAA+-type ATPases are related to peroxisome function. Pex6p functions together with Pex1p in peroxisome biogenesis, ATAD1/Msp1p plays a role in membrane protein targeting and a member of the Lon-family of proteases is associated with peroxisomal quality control. This review summarizes the current knowledge on the AAA+-proteins involved in peroxisome biogenesis and function.
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