title: Role of AAA(+)-proteins in peroxisome biogenesis and function.
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.
authors.affiliation: Abteilung für Systembiochemie, Medizinische Fakultät der Ruhr-Universität Bochum, D-44780 Bochum, Germany.
authors.forename: Immanuel
authors.initials: I
authors.lastname: Grimm
authors.affiliation: Abteilung für Systembiochemie, Medizinische Fakultät der Ruhr-Universität Bochum, D-44780 Bochum, Germany. Electronic address: ralf.erdmann@rub.de.
authors.forename: Ralf
authors.initials: R
authors.lastname: Erdmann
authors.affiliation: Abteilung für Systembiochemie, Medizinische Fakultät der Ruhr-Universität Bochum, D-44780 Bochum, Germany. Electronic address: Wolfgang.Girzalsky@rub.de.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Girzalsky
mesh_terms: D000074183:ATPases Associated with Diverse Cellular Activities; D000251:Adenosine Triphosphatases / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D005057:Eukaryotic Cells / Q000737:chemistry / Q000378:metabolism; D005786:Gene Expression Regulation; D006801:Humans; D008565:Membrane Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D001678:Organelle Biogenesis; D020675:Peroxisomes / Q000737:chemistry / Q000378:metabolism*; D010944:Plants / Q000737:chemistry / Q000378:metabolism; D020033:Protein Isoforms / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D017433:Protein Structure, Secondary; D017434:Protein Structure, Tertiary; D021381:Protein Transport; D012441:Saccharomyces cerevisiae / Q000737:chemistry / Q000378:metabolism; D029701:Saccharomyces cerevisiae Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction
chemical_list: D008565:Membrane Proteins; D020033:Protein Isoforms; D029701:Saccharomyces cerevisiae Proteins; D000251:Adenosine Triphosphatases; C000590776:MSP1 protein, S cerevisiae; D000074183:ATPases Associated with Diverse Cellular Activities; C511586:PEX1 protein, S cerevisiae; C490760:PEX6 protein, S cerevisiae
keywords: AAA(+)-ATPase; ATAD1/MSP1; Lon; Peroxisome; Pex1p; Pex6p