Gene Symbol: PEX1
Description: AAA family ATPase peroxin 1
Alias: PAS1, AAA family ATPase peroxin 1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. van der Zand A, Gent J, Braakman I, Tabak H. Biochemically distinct vesicles from the endoplasmic reticulum fuse to form peroxisomes. Cell. 2012;149:397-409 pubmed publisher
    ..Our findings demonstrate a remarkable mechanism to maintain biochemical identity of organelles by transporting crucial components via different routes to their final destination. ..
  2. Blok N, Tan D, Wang R, Penczek P, Baker D, DiMaio F, et al. Unique double-ring structure of the peroxisomal Pex1/Pex6 ATPase complex revealed by cryo-electron microscopy. Proc Natl Acad Sci U S A. 2015;112:E4017-25 pubmed publisher
    ..Here, we report structures of the Pex1/Pex6 complex; mutations in these proteins frequently cause peroxisomal diseases...
  3. Rosenkranz K, Birschmann I, Grunau S, Girzalsky W, Kunau W, Erdmann R. Functional association of the AAA complex and the peroxisomal importomer. FEBS J. 2006;273:3804-15 pubmed
  4. Saffian D, Grimm I, Girzalsky W, Erdmann R. ATP-dependent assembly of the heteromeric Pex1p-Pex6p-complex of the peroxisomal matrix protein import machinery. J Struct Biol. 2012;179:126-32 pubmed publisher
    ..Disassembly of the complex into its Pex1p and Pex6p subunits is also observed upon ATP-depletion, indicating that formation of the Pex1p/Pex6p-complex requires the presence of ATP. ..
  5. Nuttall J, Motley A, Hettema E. Deficiency of the exportomer components Pex1, Pex6, and Pex15 causes enhanced pexophagy in Saccharomyces cerevisiae. Autophagy. 2014;10:835-45 pubmed publisher
    ..We screened mutants disturbed in peroxisomal protein import, and found that a deficiency in the exportomer subunits Pex1, Pex6, and Pex15 results in enhanced turnover of peroxisomal membrane structures compared with other mutants...
  6. Gardner B, Chowdhury S, Lander G, Martin A. The Pex1/Pex6 complex is a heterohexameric AAA+ motor with alternating and highly coordinated subunits. J Mol Biol. 2015;427:1375-88 pubmed publisher
    b>Pex1 and Pex6 are Type-2 AAA+ ATPases required for the de novo biogenesis of peroxisomes. Mutations in Pex1 and Pex6 account for the majority of the most severe forms of peroxisome biogenesis disorders in humans...
  7. Ciniawsky S, Grimm I, Saffian D, Girzalsky W, Erdmann R, Wendler P. Molecular snapshots of the Pex1/6 AAA+ complex in action. Nat Commun. 2015;6:7331 pubmed publisher
    The peroxisomal proteins Pex1 and Pex6 form a heterohexameric type II AAA+ ATPase complex, which fuels essential protein transport across peroxisomal membranes...
  8. Knoops K, de Boer R, Kram A, van der Klei I. Yeast pex1 cells contain peroxisomal ghosts that import matrix proteins upon reintroduction of Pex1. J Cell Biol. 2015;211:955-62 pubmed publisher
    b>Pex1 and Pex6 are two AAA-ATPases that play a crucial role in peroxisome biogenesis...
  9. Motley A, Galvin P, Ekal L, Nuttall J, Hettema E. Reevaluation of the role of Pex1 and dynamin-related proteins in peroxisome membrane biogenesis. J Cell Biol. 2015;211:1041-56 pubmed publisher
    ..This model proposes a role in vesicle fusion for the Pex1/Pex6 complex, which has an established role in matrix protein import...

More Information


  1. Gardner B, Castanzo D, Chowdhury S, Stjepanovic G, Stefely M, Hurley J, et al. The peroxisomal AAA-ATPase Pex1/Pex6 unfolds substrates by processive threading. Nat Commun. 2018;9:135 pubmed publisher
    b>Pex1 and Pex6 form a heterohexameric motor essential for peroxisome biogenesis and function, and mutations in these AAA-ATPases cause most peroxisome-biogenesis disorders in humans...
  2. Erdmann R, Wiebel F, Flessau A, Rytka J, Beyer A, Fröhlich K, et al. PAS1, a yeast gene required for peroxisome biogenesis, encodes a member of a novel family of putative ATPases. Cell. 1991;64:499-510 pubmed
    ..cerevisiae. Here we describe the cloning, sequencing, and characterization of the PAS1 gene. Its gene product, Pas1p, has been identified as a rather hydrophilic 117 kd polypeptide...
  3. Birschmann I, Rosenkranz K, Erdmann R, Kunau W. Structural and functional analysis of the interaction of the AAA-peroxins Pex1p and Pex6p. FEBS J. 2005;272:47-58 pubmed
    ..Furthermore, we could prove that the second AAA-cassettes (D2) of both Pex1p and Pex6p were essential for peroxisomal biogenesis and thus probably comprise the overall activity of the proteins. ..
  4. Platta H, Grunau S, Rosenkranz K, Girzalsky W, Erdmann R. Functional role of the AAA peroxins in dislocation of the cycling PTS1 receptor back to the cytosol. Nat Cell Biol. 2005;7:817-22 pubmed
    ..Here we demonstrate that the AAA peroxins mediate the ATP-dependent dislocation of the peroxisomal targeting signal-1 (PTS1) receptor from the peroxisomal membrane to the cytosol. ..
  5. Debelyy M, Platta H, Saffian D, Hensel A, Thoms S, Meyer H, et al. Ubp15p, a ubiquitin hydrolase associated with the peroxisomal export machinery. J Biol Chem. 2011;286:28223-34 pubmed publisher
    ..The results merge into a picture in which removal of ubiquitin from the PTS1 receptor Pex5p is a specific event and might represent a vital step in receptor recycling...
  6. Grimm I, Saffian D, Girzalsky W, Erdmann R. Nucleotide-dependent assembly of the peroxisomal receptor export complex. Sci Rep. 2016;6:19838 pubmed publisher
    ..We reconstituted binding of the Pex1/6p-complex to Pex15p in vitro and show that Pex6p mediates binding to the cytosolic part of Pex15p via a direct ..