Gene Symbol: PEX6
Description: AAA family ATPase peroxin 6
Alias: PAS8, AAA family ATPase peroxin 6
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. Birschmann I, Stroobants A, van den Berg M, Schafer A, Rosenkranz K, Kunau W, et al. Pex15p of Saccharomyces cerevisiae provides a molecular basis for recruitment of the AAA peroxin Pex6p to peroxisomal membranes. Mol Biol Cell. 2003;14:2226-36 pubmed
    ..On the basis of these results, we propose that Pex6p exerts at least part of its function by an ATP-dependent cycle of recruitment and release to and from Pex15p. ..
  3. 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. ..
  4. 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...
  5. Seo J, Lai C, Miceli M, Jazwinski S. A novel role of peroxin PEX6: suppression of aging defects in mitochondria. Aging Cell. 2007;6:405-13 pubmed
    ..This decline has implications for stem cell aging in higher organisms. PEX6, a peroxisome biogenesis gene, has been isolated as a multicopy suppressor of an atp2 age asymmetry mutant...
  6. 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
    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...
  7. 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...
  8. 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. ..
  9. 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

More Information


  1. 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
    ..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...
  2. 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
    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...
  3. Jungwirth H, Ring J, Mayer T, Schauer A, Buttner S, Eisenberg T, et al. Loss of peroxisome function triggers necrosis. FEBS Lett. 2008;582:2882-6 pubmed publisher
    ..Here, we show that in yeast deletion of PEX6, encoding a protein involved in a key step of peroxisomal protein import, results in an increased accumulation of ..
  4. 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
    Pex1 and Pex6 are two AAA-ATPases that play a crucial role in peroxisome biogenesis...
  5. 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. ..
  6. 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...
  7. Voorn Brouwer T, van der Leij I, Hemrika W, Distel B, Tabak H. Sequence of the PAS8 gene, the product of which is essential for biogenesis of peroxisomes in Saccharomyces cerevisiae. Biochim Biophys Acta. 1993;1216:325-8 pubmed
    ..Here, we report the sequence of the complementing gene (PAS8), coding for a protein of 1030 amino acids that appears to be a novel member of the AAA-protein family which also ..
  8. 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...
  9. Grimm I, Saffian D, Girzalsky W, Erdmann R. Nucleotide-dependent assembly of the peroxisomal receptor export complex. Sci Rep. 2016;6:19838 pubmed publisher
    ..The data indicate that receptor release in peroxisomal protein import is associated with a nucleotide-depending Pex1/6p-cycle of Pex15p-binding and release. ..