Gene Symbol: Pex5
Description: peroxisomal biogenesis factor 5
Alias: PTS1-BP, PTS1R, peroxisomal targeting signal 1 receptor, PTS1 receptor, peroxin-5, peroxisomal C-terminal targeting signal import receptor, peroxisome biogenesis factor 5, peroxisome receptor 1
Species: rat
Products:     Pex5

Top Publications

  1. Baes M, Gressens P, Baumgart E, Carmeliet P, Casteels M, Fransen M, et al. A mouse model for Zellweger syndrome. Nat Genet. 1997;17:49-57 pubmed
    ..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. Braverman N, Dodt G, Gould S, Valle D. An isoform of pex5p, the human PTS1 receptor, is required for the import of PTS2 proteins into peroxisomes. Hum Mol Genet. 1998;7:1195-205 pubmed
    ..the nonsense mutation R390ter) are defective in the import of both PTS1 and PTS2 proteins, suggesting that the PTS1 receptor also mediates PTS2-targeted protein import...
  3. Dodt G, Braverman N, Wong C, Moser A, Moser H, Watkins P, et al. Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders. Nat Genet. 1995;9:115-25 pubmed
    ..Based on the observation that PXR1 exists both in the cytosol and in association with peroxisomes, we propose that PXR1 protein recognizes PTS1-containing proteins in the cytosol and directs them to the peroxisome. ..
  4. Miyata N, Okumoto K, Mukai S, Noguchi M, Fujiki Y. AWP1/ZFAND6 functions in Pex5 export by interacting with cys-monoubiquitinated Pex5 and Pex6 AAA ATPase. Traffic. 2012;13:168-83 pubmed publisher
    During biogenesis of the peroxisome, a subcellular organelle, the peroxisomal-targeting signal 1 (PTS1) receptor Pex5 functions as a shuttling receptor for PTS1-containing peroxisomal matrix proteins...
  5. Harano T, Nose S, Uezu R, Shimizu N, Fujiki Y. Hsp70 regulates the interaction between the peroxisome targeting signal type 1 (PTS1)-receptor Pex5p and PTS1. Biochem J. 2001;357:157-65 pubmed
  6. Williams C, Schueller N, Thompson C, van den Berg M, van Haren S, Erdmann R, et al. The Peroxisomal Targeting Signal 1 in sterol carrier protein 2 is autonomous and essential for receptor recognition. BMC Biochem. 2011;12:12 pubmed publisher
    ..In this study, we have tested the contribution of a second, ancillary receptor-cargo binding site, which was found in addition to the characterised Peroxisomal Target Signal type 1...
  7. Oliveira M, Gouveia A, Pinto R, Sa Miranda C, Azevedo J. The energetics of Pex5p-mediated peroxisomal protein import. J Biol Chem. 2003;278:39483-8 pubmed
    ..This observation raises the possibility that at the peroxisomal membrane ATP is needed predominantly (if not exclusively) downstream of the protein translocation step to reset the Pex5p-mediated transport system. ..
  8. Yamashita H, Avraham S, Jiang S, London R, Van Veldhoven P, Subramani S, et al. Characterization of human and murine PMP20 peroxisomal proteins that exhibit antioxidant activity in vitro. J Biol Chem. 1999;274:29897-904 pubmed
    ..HsPMP20 bound directly to the human peroxisomal targeting signal 1 receptor, HsPEX5...
  9. Shiozawa K, Konarev P, Neufeld C, Wilmanns M, Svergun D. Solution structure of human Pex5.Pex14.PTS1 protein complexes obtained by small angle X-ray scattering. J Biol Chem. 2009;284:25334-42 pubmed publisher
    ..These results suggest that the extended structure of Pex5p may play a role in interactions with other substrates such as lipids and membrane proteins during the formation of functional multiprotein complexes. ..

More Information


  1. Dirkx R, Meyhi E, Asselberghs S, Reddy J, Baes M, Van Veldhoven P. Beta-oxidation in hepatocyte cultures from mice with peroxisomal gene knockouts. Biochem Biophys Res Commun. 2007;357:718-23 pubmed
    ..More surprisingly, dicarboxylic fatty acid oxidation was impaired in MFP-1 but not in MFP-2 knockout hepatocytes, indicating that MFP-1 might play more than an obsolete role in peroxisomal beta-oxidation. ..
  2. Reguenga C, Oliveira M, Gouveia A, Sa Miranda C, Azevedo J. Characterization of the mammalian peroxisomal import machinery: Pex2p, Pex5p, Pex12p, and Pex14p are subunits of the same protein assembly. J Biol Chem. 2001;276:29935-42 pubmed
    ..Taken together, our data indicate that Pex2p, Pex5p, Pex12p, and Pex14p, on one side, and Pex13p, on the other, are subunits of two stable protein complexes that probably interact with each other in the peroxisomal membrane. ..
  3. Vanhorebeek I, Baes M, Declercq P. Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model. Biochim Biophys Acta. 2001;1532:28-36 pubmed
    ..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. Amery L, Sano H, Mannaerts G, Snider J, Van Looy J, Fransen M, et al. Identification of PEX5p-related novel peroxisome-targeting signal 1 (PTS1)-binding proteins in mammals. Biochem J. 2001;357:635-46 pubmed
    ..PEX5Rp did not bind to Pex14p or to the RING finger motif of Pex12p, and could not restore PTS1 protein import in Pex5(-/-) mouse fibroblasts...
  5. Neufeld C, Filipp F, Simon B, Neuhaus A, Schüller N, David C, et al. Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19. EMBO J. 2009;28:745-54 pubmed publisher
    ..Pex14 is a central component of the peroxisomal import machinery and binds the soluble receptors Pex5 and Pex19, which have important function in the assembly of peroxisome matrix and membrane, respectively...
  6. Fransen M, Amery L, Hartig A, Brees C, Rabijns A, Mannaerts G, et al. Comparison of the PTS1- and Rab8b-binding properties of Pex5p and Pex5Rp/TRIP8b. Biochim Biophys Acta. 2008;1783:864-73 pubmed publisher
    ..employed various methodological approaches to investigate and compare the binding specificities of the human PTS1 receptor Pex5p and the related protein Pex5Rp/TRIP8b towards a subset of targets, including Rab8b and various C-termini ..
  7. Baumgart E, Vanhorebeek I, Grabenbauer M, Borgers M, Declercq P, Fahimi H, et al. Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse). Am J Pathol. 2001;159:1477-94 pubmed
    ..organ dysfunctions in Zellweger syndrome, we have recently developed a knockout-mouse model by disrupting the PEX5 gene, encoding the targeting receptor for most peroxisomal matrix proteins (M Baes, P Gressens, E Baumgart, P ..
  8. Dammai V, Subramani S. The human peroxisomal targeting signal receptor, Pex5p, is translocated into the peroxisomal matrix and recycled to the cytosol. Cell. 2001;105:187-96 pubmed
    ..This unusual shuttling mechanism for the PTS1 receptor distinguishes protein import into peroxisomes from that into most other organelles, with the exception of the ..
  9. Chang C, Warren D, Sacksteder K, Gould S. PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import. J Cell Biol. 1999;147:761-74 pubmed
    ..and coimmunoprecipitation experiments, we observed that the zinc-binding domain of PEX12 binds both PEX5, the PTS1 receptor, and PEX10, another integral peroxisomal membrane protein required for peroxisomal matrix protein import...
  10. Bjorkman J, Gould S, Crane D. Pex13, the mouse ortholog of the human peroxisome biogenesis disorder PEX13 gene: gene structure, tissue expression, and localization of the protein to peroxisomes. Genomics. 2002;79:162-8 pubmed
    ..We infer from these findings that targeted disruption of mouse Pex13 would provide an appropriate model for the study of PEX13 dysfunction in humans. ..
  11. Janssen A, Gressens P, Grabenbauer M, Baumgart E, Schad A, Vanhorebeek I, et al. Neuronal migration depends on intact peroxisomal function in brain and in extraneuronal tissues. J Neurosci. 2003;23:9732-41 pubmed
    ..Peroxisomes were selectively reconstituted either in brain or liver of Pex5 knock-out mice, a model for Zellweger syndrome, by tissue-selective overexpression of Pex5p...
  12. Costa Rodrigues J, Carvalho A, Gouveia A, Fransen M, Sa Miranda C, Azevedo J. The N terminus of the peroxisomal cycling receptor, Pex5p, is required for redirecting the peroxisome-associated peroxin back to the cytosol. J Biol Chem. 2004;279:46573-9 pubmed
    ..Our data indicate that the export step of Pex5p from the peroxisomal compartment (in contrast with its insertion into the organelle membrane) is highly dependent on the temperature. ..
  13. Ghys K, Fransen M, Mannaerts G, Van Veldhoven P. Functional studies on human Pex7p: subcellular localization and interaction with proteins containing a peroxisome-targeting signal type 2 and other peroxins. Biochem J. 2002;365:41-50 pubmed
    ..The GFP fusion proteins, tagged at either the N- or C-terminus, were able to restore PTS2 import in rhizomelic chondrodysplasia punctata fibroblasts, and Pex7p-GFP was located both in the lumen of peroxisomes and in the cytosol...
  14. Amery L, Mannaerts G, Subramani S, Van Veldhoven P, Fransen M. Identification of a novel human peroxisomal 2,4-dienoyl-CoA reductase related protein using the M13 phage protein VI phage display technology. Comb Chem High Throughput Screen. 2001;4:545-52 pubmed
    ..These results further demonstrate the suitability of the gVI-cDNA phage display technology for cDNA expression cloning using an antibody as a probe. ..
  15. Dirkx R, Vanhorebeek I, Martens K, Schad A, Grabenbauer M, Fahimi D, et al. Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities. Hepatology. 2005;41:868-78 pubmed
    ..the present study, a mouse model with hepatocyte-selective elimination of peroxisomes was generated by inbreeding Pex5-loxP and albumin-Cre mice to investigate the consequences of peroxisome deletion on the functioning of hepatocytes...
  16. Huyghe S, Schmalbruch H, De Gendt K, Verhoeven G, Guillou F, Van Veldhoven P, et al. Peroxisomal multifunctional protein 2 is essential for lipid homeostasis in Sertoli cells and male fertility in mice. Endocrinology. 2006;147:2228-36 pubmed
    ..mice with a Sertoli cell-selective elimination of peroxisomes through cell type-specific inactivation of the peroxin 5 gene...
  17. Freitas M, Francisco T, Rodrigues T, Alencastre I, Pinto M, Grou C, et al. PEX5 protein binds monomeric catalase blocking its tetramerization and releases it upon binding the N-terminal domain of PEX14. J Biol Chem. 2011;286:40509-19 pubmed publisher
    Newly synthesized peroxisomal matrix proteins are targeted to the organelle by PEX5. PEX5 has a dual role in this process. First, it acts as a soluble receptor recognizing these proteins in the cytosol...
  18. Harper C, Berg J, Gould S. PEX5 binds the PTS1 independently of Hsp70 and the peroxin PEX12. J Biol Chem. 2003;278:7897-901 pubmed
    ..b>PEX5 binds the PTS1 through its C-terminal 40-kDa tetratricopeptide repeat domain and is essential for import of PTS1-..
  19. Carvalho A, Pinto M, Grou C, Alencastre I, Fransen M, Sa Miranda C, et al. Ubiquitination of mammalian Pex5p, the peroxisomal import receptor. J Biol Chem. 2007;282:31267-72 pubmed
    ..Together with mechanistic data recently described for yeast Pex5p, these findings provide strong evidence for the existence of Pex4p- and Pex22p-like proteins in mammals. ..
  20. Jones J, Morrell J, Gould S. PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins. J Cell Biol. 2004;164:57-67 pubmed
  21. Amery L, Fransen M, De Nys K, Mannaerts G, Van Veldhoven P. Mitochondrial and peroxisomal targeting of 2-methylacyl-CoA racemase in humans. J Lipid Res. 2000;41:1752-9 pubmed
    ..From the in vitro interaction between recombinant racemase and recombinant human PTS1 receptor (Pex5p), and the peroxisomal localization of green fluorescent protein (GFP) fused to the N terminus of full-..
  22. Janssen A, Baes M, Gressens P, Mannaerts G, Declercq P, Van Veldhoven P. Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice. Lab Invest. 2000;80:31-5 pubmed
    ..The purpose of this study was to investigate (a) whether DHA levels were also reduced in newborn PEX5 knockout mice, the mouse model of Zellweger syndrome that we recently generated; (b) whether these levels could be ..
  23. Grou C, Francisco T, Rodrigues T, Freitas M, Pinto M, Carvalho A, et al. Identification of ubiquitin-specific protease 9X (USP9X) as a deubiquitinase acting on ubiquitin-peroxin 5 (PEX5) thioester conjugate. J Biol Chem. 2012;287:12815-27 pubmed publisher
    b>Peroxin 5 (PEX5), the peroxisomal protein shuttling receptor, binds newly synthesized peroxisomal matrix proteins in the cytosol and promotes their translocation across the organelle membrane...