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Biochimica et biophysica acta, 2016-05, Vol.1863 (5), p.828-837
2016
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Autor(en) / Beteiligte
Titel
Role of AAA+-proteins in peroxisome biogenesis and function
Ist Teil von
  • Biochimica et biophysica acta, 2016-05, Vol.1863 (5), p.828-837
Ort / Verlag
Netherlands: Elsevier B.V
Erscheinungsjahr
2016
Quelle
MEDLINE
Beschreibungen/Notizen
  • 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. This article is part of a Special Issue entitled: Peroxisomes edited by Ralf Erdmann. •Four peroxisomal AAA+-proteins are known: Pex1p, Pex6p, ATAD1/Msp1p and Lon.•Pex1p/Pex6p form a hexameric complex required for peroxisomal protein import.•ATAD1/Msp1p plays a role in targeting of mitochondrial tail-anchored proteins, the function in peroxisomes is unknown.•Lon is an AAA-protease required for the quality control of peroxisomal matrix proteins.

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