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Gene Review

PEX1  -  AAA family ATPase peroxin 1

Saccharomyces cerevisiae S288c

Synonyms: PAS1, Peroxin-1, Peroxisomal ATPase PEX1, Peroxisomal assembly protein 1, Peroxisome biogenesis protein PAS1, ...
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Disease relevance of PEX1


High impact information on PEX1

  • Pex14p, an S. cerevisiae peroxin, is attached to the outer face of the peroxisomal membrane and is a component of the protein import machinery [2].
  • Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders [3].
  • These data demonstrate an important role for PEX1 in peroxisome biogenesis and suggest that mutations in this gene are the most common cause of the PBDs [3].
  • Expression of PEX1 rescued the cells from the biogenesis defect in human fibroblasts of complementation group 1 (CG1), the largest PBD complementation group [4].
  • Human PEX1 has been identified by computer-based 'homology probing' using the ScPex1p sequence to screen databases of expressed sequence tags (dbEST) for human cDNA clones [4].

Biological context of PEX1


Anatomical context of PEX1

  • As has been shown previously for P. pastoris pex1, pex6, and pex22 mutant cells, we show here that pex4Delta mutant cells contain peroxisomal membrane protein-containing peroxisomes that import residual amounts of peroxisomal matrix proteins [10].
  • Pex15p of Saccharomyces cerevisiae provides a molecular basis for recruitment of the AAA peroxin Pex6p to peroxisomal membranes [11].
  • Within this context, we discuss the proposed role for the endoplasmic reticulum in the formation of preperoxisomes and the potential for 15 Arabidopsis peroxin homologs to function in the biogenesis of peroxisomes in plant cells [12].
  • In transiently transformed Arabidopsis and tobacco BY-2 suspension-cultured cells, epitope-tagged AtPex3p (form 2) sorted post-translationally from the cytosol directly to peroxisomes, the first sorting pathway described for any peroxin in plants [13].

Associations of PEX1 with chemical compounds


Physical interactions of PEX1


Other interactions of PEX1

  • Northern hybridization experiments further demonstrated that expression of two genes encoding enzymes of peroxisomal beta-oxidation (beta-ketothiolase, trifunctional enzyme) and of a gene involved in peroxisome assembly (PAS1) is also negatively affected by the adr1 null mutation [20].
  • PEX11 codes for a peroxin that is critical for normal peroxisome proliferation, and its promoter was shown similarly to contain a UAS1-like element overlapping the ORE [21].
  • AFG1, a new member of the SEC18-NSF, PAS1, CDC48-VCP, TBP family of ATPases [22].
  • The PAS5 gene was cloned and found to encode a 127-kD protein, which contains a 200-amino acid-long region of homology with PAS1, NEM-sensitive factor (NSF), and other related ATPases [23].
  • Ubiquitination of Pex5p is shown to take place exclusively at the peroxisomal membrane after the docking step, and requires the presence of the RING-finger peroxin Pex10p [24].

Analytical, diagnostic and therapeutic context of PEX1


  1. Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I. Tamura, S., Okumoto, K., Toyama, R., Shimozawa, N., Tsukamoto, T., Suzuki, Y., Osumi, T., Kondo, N., Fujiki, Y. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  2. Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways. Albertini, M., Rehling, P., Erdmann, R., Girzalsky, W., Kiel, J.A., Veenhuis, M., Kunau, W.H. Cell (1997) [Pubmed]
  3. Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders. Reuber, B.E., Germain-Lee, E., Collins, C.S., Morrell, J.C., Ameritunga, R., Moser, H.W., Valle, D., Gould, S.J. Nat. Genet. (1997) [Pubmed]
  4. Human PEX1 is mutated in complementation group 1 of the peroxisome biogenesis disorders. Portsteffen, H., Beyer, A., Becker, E., Epplen, C., Pawlak, A., Kunau, W.H., Dodt, G. Nat. Genet. (1997) [Pubmed]
  5. Oxygen and haem regulate the synthesis of peroxisomal proteins: catalase A, acyl-CoA oxidase and Pex1p in the yeast Saccharomyces cerevisiae; the regulation of these proteins by oxygen is not mediated by haem. Skoneczny, M., Rytka, J. Biochem. J. (2000) [Pubmed]
  6. Pex17p of Saccharomyces cerevisiae is a novel peroxin and component of the peroxisomal protein translocation machinery. Huhse, B., Rehling, P., Albertini, M., Blank, L., Meller, K., Kunau, W.H. J. Cell Biol. (1998) [Pubmed]
  7. NVL: a new member of the AAA family of ATPases localized to the nucleus. Germain-Lee, E.L., Obie, C., Valle, D. Genomics (1997) [Pubmed]
  8. YHR150w and YDR479c encode peroxisomal integral membrane proteins involved in the regulation of peroxisome number, size, and distribution in Saccharomyces cerevisiae. Vizeacoumar, F.J., Torres-Guzman, J.C., Tam, Y.Y., Aitchison, J.D., Rachubinski, R.A. J. Cell Biol. (2003) [Pubmed]
  9. The yeast YKL741 gene situated on the left arm of chromosome XI codes for a homologue of the human ALD protein. Bossier, P., Fernandes, L., Vilela, C., Rodrigues-Pousada, C. Yeast (1994) [Pubmed]
  10. The peroxisome biogenesis factors pex4p, pex22p, pex1p, and pex6p act in the terminal steps of peroxisomal matrix protein import. Collins, C.S., Kalish, J.E., Morrell, J.C., McCaffery, J.M., Gould, S.J. Mol. Cell. Biol. (2000) [Pubmed]
  11. Pex15p of Saccharomyces cerevisiae provides a molecular basis for recruitment of the AAA peroxin Pex6p to peroxisomal membranes. Birschmann, I., Stroobants, A.K., van den Berg, M., Schäfer, A., Rosenkranz, K., Kunau, W.H., Tabak, H.F. Mol. Biol. Cell (2003) [Pubmed]
  12. How are peroxisomes formed? The role of the endoplasmic reticulum and peroxins. Mullen, R.T., Flynn, C.R., Trelease, R.N. Trends Plant Sci. (2001) [Pubmed]
  13. Sorting pathway and molecular targeting signals for the Arabidopsis peroxin 3. Hunt, J.E., Trelease, R.N. Biochem. Biophys. Res. Commun. (2004) [Pubmed]
  14. A novel family of longer chain length dolichols present in oleate-induced yeast Saccharomyces cerevisiae. Szkopinska, A., Swiezewska, E., Skoneczny, M. Biochimie (2001) [Pubmed]
  15. Identification and functional characterization of Arabidopsis PEROXIN4 and the interacting protein PEROXIN22. Zolman, B.K., Monroe-Augustus, M., Silva, I.D., Bartel, B. Plant Cell (2005) [Pubmed]
  16. Effect of site-directed mutagenesis of conserved lysine residues upon Pas1 protein function in peroxisome biogenesis. Krause, T., Kunau, W.H., Erdmann, R. Yeast (1994) [Pubmed]
  17. Pex8p, an intraperoxisomal peroxin of Saccharomyces cerevisiae required for protein transport into peroxisomes binds the PTS1 receptor pex5p. Rehling, P., Skaletz-Rorowski, A., Girzalsky, W., Voorn-Brouwer, T., Franse, M.M., Distel, B., Veenhuis, M., Kunau, W.H., Erdmann, R. J. Biol. Chem. (2000) [Pubmed]
  18. Arabidopsis PEX19 is a dimeric protein that binds the peroxin PEX10. Hadden, D.A., Phillipson, B.A., Johnston, K.A., Brown, L.A., Manfield, I.W., El-Shami, M., Sparkes, I.A., Baker, A. Mol. Membr. Biol. (2006) [Pubmed]
  19. Acyl-CoA oxidase is imported as a heteropentameric, cofactor-containing complex into peroxisomes of Yarrowia lipolytica. Titorenko, V.I., Nicaud, J.M., Wang, H., Chan, H., Rachubinski, R.A. J. Cell Biol. (2002) [Pubmed]
  20. The Saccharomyces cerevisiae ADR1 gene is a positive regulator of transcription of genes encoding peroxisomal proteins. Simon, M., Adam, G., Rapatz, W., Spevak, W., Ruis, H. Mol. Cell. Biol. (1991) [Pubmed]
  21. Saccharomyces cerevisiae Adr1p governs fatty acid beta-oxidation and peroxisome proliferation by regulating POX1 and PEX11. Gurvitz, A., Hiltunen, J.K., Erdmann, R., Hamilton, B., Hartig, A., Ruis, H., Rottensteiner, H. J. Biol. Chem. (2001) [Pubmed]
  22. AFG1, a new member of the SEC18-NSF, PAS1, CDC48-VCP, TBP family of ATPases. Lee, Y.J., Wickner, R.B. Yeast (1992) [Pubmed]
  23. Cloning and characterization of PAS5: a gene required for peroxisome biogenesis in the methylotrophic yeast Pichia pastoris. Spong, A.P., Subramani, S. J. Cell Biol. (1993) [Pubmed]
  24. Ubiquitination of the peroxisomal import receptor Pex5p. Platta, H.W., Girzalsky, W., Erdmann, R. Biochem. J. (2004) [Pubmed]
  25. Analysis of mammalian peroxin interactions using a non-transcription-based bacterial two-hybrid assay. Fransen, M., Brees, C., Ghys, K., Amery, L., Mannaerts, G.P., Ladant, D., Van Veldhoven, P.P. Mol. Cell Proteomics (2002) [Pubmed]
  26. A new self-assembled peroxisomal vesicle required for efficient resealing of the plasma membrane. Jedd, G., Chua, N.H. Nat. Cell Biol. (2000) [Pubmed]
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