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Gene Review

PEX13  -  peroxisomal biogenesis factor 13

Homo sapiens

Synonyms: NALD, PBD11A, PBD11B, Peroxin-13, Peroxisomal membrane protein PEX13, ...
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Disease relevance of PEX13


High impact information on PEX13


Biological context of PEX13


Anatomical context of PEX13

  • We examined the ability of PEX13 expression to rescue the peroxisomal protein import defects of fibroblast cells representing all known PBD complementation groups [8].
  • Therefore, ZP128 and ZP150 are CHO cell lines with a phenotype of impaired PEX13 [7].
  • The strong interaction of Pex5p with the membrane of the organelle is not affected by mild protease treatment of intact organelles, conditions that result in the partial degradation of Pex13p [10].

Associations of PEX13 with chemical compounds

  • Alanine-scanning analysis of the highly conserved seven (six in Pex5pS) pentapeptide WXXXF/Y motifs residing at the N-terminal region indicated that these motifs were essential for the interaction of Pex5p with Pex14p and Pex13p [11].
  • ZWS activity was decreased by treatment with polymyxin B, but not with lysozyme and zymolyase [2].

Physical interactions of PEX13

  • PEX13 protein has an SH3 docking site that binds to the PTS-1 receptor [12].
  • Furthermore, we detected the Pex13p complexes likewise formed with Pex5pL-bound Pex7p-PTS2 proteins [13].
  • Here we demonstrate that a mutant of Pex13p that fails to bind to Pex19p nevertheless targets to and integrates into the peroxisomal membrane [14].

Other interactions of PEX13

  • We have previously reported the identification of human PEX13, the gene encoding the docking factor for the PTS1 receptor, or PEX5 protein [8].
  • Genomic structure of PEX13, a candidate peroxisome biogenesis disorder gene [8].
  • Import of matrix proteins into peroxisomes requires two targeting signal-specific import receptors, Pex5p and Pex7p, and their binding partners at the peroxisomal membrane, Pex13p and Pex14p [15].
  • Localization to peroxisomes of membrane proteins such as Pex14p, Pex13p, and PMP70 was interfered with in CHO-K1 cells by a higher level expression of the pex16 patient-derived dysfunctional but topogenically active Pex16pR176ter comprising resides 1-176 or of the C-terminal cytoplasmic part starting from residues at 244 to the C terminus [16].
  • Pex5p carrying the cargos, PTS1 and PTS2, docks with the initial site Pex14p in a putative import machinery, subsequently translocating to other components such as Pex13p, Pex2p, Pex10p and Pex12p, whereby the matrix proteins are imported [17].

Analytical, diagnostic and therapeutic context of PEX13

  • To further examine the molecular mechanism underlying this competition, six evolutionarily conserved amino acids in the Pex5p/Pex13p/Pex19p binding domain of Pex14p were subjected to site-directed mutagenesis and the corresponding mutants functionally analyzed [14].


  1. Pex13, the mouse ortholog of the human peroxisome biogenesis disorder PEX13 gene: gene structure, tissue expression, and localization of the protein to peroxisomes. Björkman, J., Gould, S.J., Crane, D.I. Genomics (2002) [Pubmed]
  2. Activation of toll-like receptor-mediated NF-kappa beta by zymosan-derived water-soluble fraction: possible contribution of endotoxin-like substances. Ikeda, Y., Adachi, Y., Ishibashi, K., Miura, N., Ohno, N. Immunopharmacology and immunotoxicology. (2005) [Pubmed]
  3. Ultrasonic evaluation of portosystemic collateral circulation in portal hypertension. Sheth, S.G., Amarapurkar, D.N., Chopra, K.B., Mani, S.A., Mehta, P.J. The Journal of the Association of Physicians of India. (1996) [Pubmed]
  4. The SH3 domain of the Saccharomyces cerevisiae peroxisomal membrane protein Pex13p functions as a docking site for Pex5p, a mobile receptor for the import PTS1-containing proteins. Elgersma, Y., Kwast, L., Klein, A., Voorn-Brouwer, T., van den Berg, M., Metzig, B., America, T., Tabak, H.F., Distel, B. J. Cell Biol. (1996) [Pubmed]
  5. Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype. Maxwell, M., Bjorkman, J., Nguyen, T., Sharp, P., Finnie, J., Paterson, C., Tonks, I., Paton, B.C., Kay, G.F., Crane, D.I. Mol. Cell. Biol. (2003) [Pubmed]
  6. Nonsense and temperature-sensitive mutations in PEX13 are the cause of complementation group H of peroxisome biogenesis disorders. Shimozawa, N., Suzuki, Y., Zhang, Z., Imamura, A., Toyama, R., Mukai, S., Fujiki, Y., Tsukamoto, T., Osumi, T., Orii, T., Wanders, R.J., Kondo, N. Hum. Mol. Genet. (1999) [Pubmed]
  7. Isolation, characterization and mutation analysis of PEX13-defective Chinese hamster ovary cell mutants. Toyama, R., Mukai, S., Itagaki, A., Tamura, S., Shimozawa, N., Suzuki, Y., Kondo, N., Wanders, R.J., Fujiki, Y. Hum. Mol. Genet. (1999) [Pubmed]
  8. Genomic structure of PEX13, a candidate peroxisome biogenesis disorder gene. Björkman, J., Stetten, G., Moore, C.S., Gould, S.J., Crane, D.I. Genomics (1998) [Pubmed]
  9. Pex13p: Docking or cargo handling protein? Williams, C., Distel, B. Biochim. Biophys. Acta (2006) [Pubmed]
  10. Characterization of peroxisomal Pex5p from rat liver. Pex5p in the Pex5p-Pex14p membrane complex is a transmembrane protein. Gouveia, A.M., Reguenga, C., Oliveira, M.E., Sa-Miranda, C., Azevedo, J.E. J. Biol. Chem. (2000) [Pubmed]
  11. Peroxisomal targeting signal receptor Pex5p interacts with cargoes and import machinery components in a spatiotemporally differentiated manner: conserved Pex5p WXXXF/Y motifs are critical for matrix protein import. Otera, H., Setoguchi, K., Hamasaki, M., Kumashiro, T., Shimizu, N., Fujiki, Y. Mol. Cell. Biol. (2002) [Pubmed]
  12. Clinical, biochemical and genetic aspects and neuronal migration in peroxisome biogenesis disorders. Suzuki, Y., Shimozawa, N., Imamura, A., Fukuda, S., Zhang, Z., Orii, T., Kondo, N. J. Inherit. Metab. Dis. (2001) [Pubmed]
  13. Molecular Mechanisms of Import of Peroxisome-targeting Signal Type 2 (PTS2) Proteins by PTS2 Receptor Pex7p and PTS1 Receptor Pex5pL. Mukai, S., Fujiki, Y. J. Biol. Chem. (2006) [Pubmed]
  14. Potential role for Pex19p in assembly of PTS-receptor docking complexes. Fransen, M., Vastiau, I., Brees, C., Brys, V., Mannaerts, G.P., Van Veldhoven, P.P. J. Biol. Chem. (2004) [Pubmed]
  15. Recombinant human peroxisomal targeting signal receptor PEX5. Structural basis for interaction of PEX5 with PEX14. Schliebs, W., Saidowsky, J., Agianian, B., Dodt, G., Herberg, F.W., Kunau, W.H. J. Biol. Chem. (1999) [Pubmed]
  16. The membrane biogenesis peroxin Pex16p. Topogenesis and functional roles in peroxisomal membrane assembly. Honsho, M., Hiroshige, T., Fujiki, Y. J. Biol. Chem. (2002) [Pubmed]
  17. Peroxisome biogenesis and peroxisome biogenesis disorders. Fujiki, Y. FEBS Lett. (2000) [Pubmed]
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