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Gene Review

Pex5  -  peroxisomal biogenesis factor 5

Mus musculus

Synonyms: AW212715, ESTM1, PTS1 receptor, PTS1-BP, PTS1R, ...
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Disease relevance of Pex5

  • The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation [1].

High impact information on Pex5

  • Pxr1-/- mice lacked morphologically identifiable peroxisomes and exhibited the typical biochemical abnormalities of Zellweger patients [2].
  • Subsequent analysis of extracts from fibroblasts of Pex5-/- mice and mice with a defective peroxisomal beta-oxidation (lacking D-specific multifunctional protein 2 (MFP2)), revealed, again, a similar rise in this particular ceramide [3].
  • Because several studies indicated that peroxisomes are important for the biosynthesis of isoprenoids, we wanted to investigate whether a reduced availability of isoprenoids could be one of the pathogenic factors contributing to the severe phenotype of the Pex5(-/-) mouse, a model for Zellweger syndrome [4].
  • No significant difference was observed between the cholesterol production rates of Pex5(-/-) and normal fibroblasts [4].
  • The hepatic ubiquinone content was found to be even higher in Pex5(-/-) mice as compared to wild type or heterozygous littermates [4].

Associations of Pex5 with chemical compounds

  • To investigate whether the Pex5(-/-) fetuses are able to synthesise their own isoprenoids, fibroblasts derived from these mice were incubated with radiolabeled mevalonolactone as a substrate for isoprenoid synthesis [4].

Analytical, diagnostic and therapeutic context of Pex5

  • We generated a Zellweger animal model through inactivation of the murine Pxr1 gene (formally known as Pex5) that encodes the import receptor for most peroxisomal matrix proteins [2].


  1. The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation. Baes, M., Gressens, P., Huyghe, S., De, N.K., Qi, C., Jia, Y., Mannaerts, G.P., Evrard, P., Van, V.P., Declercq, P.E., Reddy, J.K. J. Neuropathol. Exp. Neurol. (2002) [Pubmed]
  2. A mouse model for Zellweger syndrome. Baes, M., Gressens, P., Baumgart, E., Carmeliet, P., Casteels, M., Fransen, M., Evrard, P., Fahimi, D., Declercq, P.E., Collen, D., van Veldhoven, P.P., Mannaerts, G.P. Nat. Genet. (1997) [Pubmed]
  3. Mass spectrometric analysis of ceramide perturbations in brain and fibroblasts of mice and human patients with peroxisomal disorders. Pettus, B.J., Baes, M., Busman, M., Hannun, Y.A., Van Veldhoven, P.P. Rapid Commun. Mass Spectrom. (2004) [Pubmed]
  4. Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model. Vanhorebeek, I., Baes, M., Declercq, P.E. Biochim. Biophys. Acta (2001) [Pubmed]
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