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Gene Review

PEX7  -  Pex7p

Saccharomyces cerevisiae S288c

Synonyms: PAS7, PEB1, PTS2 receptor, Peroxin-7, Peroxisomal targeting signal 2 receptor, ...
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Disease relevance of PEX7

  • We investigated the molecular dysfunction of Pex7p variants in mammals, including Pex7p-W221ter and Pex7p with one site mutation at G217R, A218V, or L292ter, which frequently occurs in the human fatal genetic peroxisomal disease rhizomelic chondrodysplasia punctata, showing a cell phenotype of PTS2 import defect [1].
  • Our observation that Pas7p also interacts with the human peroxisomal thiolase suggests that in the human peroxisomal disorders characterized by an import defect for PTS2 proteins (classical rhizomelic chondrodysplasia punctata), a functional homologue of Pas7p may be impaired [2].

High impact information on PEX7


Biological context of PEX7


Anatomical context of PEX7

  • Subsequently, the formed Pex7p/cargo complex is transported from the cytosol to the peroxisomal docking complex, consisting of Pex13p and Pex14p [9].
  • Subcellular fractionation and protease protection studies demonstrated bimodal distribution of Pex7p between the cytoplasm and peroxisomes in CHO and human cells [1].
  • We suggest that Pas7p mediates the binding of thiolase to these organelles [7].
  • Interference between the PHA-4 and PEB-1 transcription factors in formation of the Caenorhabditis elegans pharynx [10].
  • Here we describe the novel factor PEB-1, which is coexpressed with PHA-4 in many pharyngeal cell types, including muscles, epithelial cells, marginal cells, and glands, but is undetectable in the pharyngeal nervous system [11].

Associations of PEX7 with chemical compounds

  • Thus the NH2-terminal 56-amino acid residues of Peb1p are necessary and sufficient for peroxisomal targeting [12].
  • We have cloned PAS7 by complementation of the oleic acid non-utilizing phenotype of the pas7-1 strain [7].

Physical interactions of PEX7

  • We also found that the cytosolic Pex7p/thiolase-containing complex includes Pex18p [9].

Other interactions of PEX7


Analytical, diagnostic and therapeutic context of PEX7


  1. Intracellular localization, function, and dysfunction of the peroxisome-targeting signal type 2 receptor, Pex7p, in mammalian cells. Mukai, S., Ghaedi, K., Fujiki, Y. J. Biol. Chem. (2002) [Pubmed]
  2. The import receptor for the peroxisomal targeting signal 2 (PTS2) in Saccharomyces cerevisiae is encoded by the PAS7 gene. Rehling, P., Marzioch, M., Niesen, F., Wittke, E., Veenhuis, M., Kunau, W.H. EMBO J. (1996) [Pubmed]
  3. Rhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptor. Purdue, P.E., Zhang, J.W., Skoneczny, M., Lazarow, P.B. Nat. Genet. (1997) [Pubmed]
  4. Pex8p: an intraperoxisomal organizer of the peroxisomal import machinery. Agne, B., Meindl, N.M., Niederhoff, K., Einwächter, H., Rehling, P., Sickmann, A., Meyer, H.E., Girzalsky, W., Kunau, W.H. Mol. Cell (2003) [Pubmed]
  5. Pex7p translocates in and out of peroxisomes in Saccharomyces cerevisiae. Nair, D.M., Purdue, P.E., Lazarow, P.B. J. Cell Biol. (2004) [Pubmed]
  6. Yeast Pex14p possesses two functionally distinct Pex5p and one Pex7p binding sites. Niederhoff, K., Meindl-Beinker, N.M., Kerssen, D., Perband, U., Schäfer, A., Schliebs, W., Kunau, W.H. J. Biol. Chem. (2005) [Pubmed]
  7. PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomes. Marzioch, M., Erdmann, R., Veenhuis, M., Kunau, W.H. EMBO J. (1994) [Pubmed]
  8. Formation of the peroxisome lumen is abolished by loss of Pichia pastoris Pas7p, a zinc-binding integral membrane protein of the peroxisome. Kalish, J.E., Theda, C., Morrell, J.C., Berg, J.M., Gould, S.J. Mol. Cell. Biol. (1995) [Pubmed]
  9. Interactions of Pex7p and Pex18p/Pex21p with the peroxisomal docking machinery: implications for the first steps in PTS2 protein import. Stein, K., Schell-Steven, A., Erdmann, R., Rottensteiner, H. Mol. Cell. Biol. (2002) [Pubmed]
  10. Interference between the PHA-4 and PEB-1 transcription factors in formation of the Caenorhabditis elegans pharynx. Kalb, J.M., Beaster-Jones, L., Fernandez, A.P., Okkema, P.G., Goszczynski, B., McGhee, J.D. J. Mol. Biol. (2002) [Pubmed]
  11. The Caenorhabditis elegans peb-1 gene encodes a novel DNA-binding protein involved in morphogenesis of the pharynx, vulva, and hindgut. Thatcher, J.D., Fernandez, A.P., Beaster-Jones, L., Haun, C., Okkema, P.G. Dev. Biol. (2001) [Pubmed]
  12. Peb1p (Pas7p) is an intraperoxisomal receptor for the NH2-terminal, type 2, peroxisomal targeting sequence of thiolase: Peb1p itself is targeted to peroxisomes by an NH2-terminal peptide. Zhang, J.W., Lazarow, P.B. J. Cell Biol. (1996) [Pubmed]
  13. 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]
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