Gene Symbol: PEX5
Description: peroxisomal biogenesis factor 5
Alias: PBD2A, PBD2B, PTS1-BP, PTS1R, PXR1, RCDP5, peroxisomal biogenesis factor 5, PTS1 receptor, peroxin-5, peroxisomal C-terminal targeting signal import receptor, peroxisomal import receptor 5, peroxisomal targeting signal 1 (SKL type) receptor, peroxisomal targeting signal 1 receptor, peroxisomal targeting signal import receptor, peroxisomal targeting signal receptor 1, peroxisome receptor 1
Species: human
Products:     PEX5

Top Publications

  1. Wiemer E, Nuttley W, Bertolaet B, Li X, Francke U, Wheelock M, et al. Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders. J Cell Biol. 1995;130:51-65 pubmed
    ..We have cloned and sequenced the human cDNA homologue (PTS1R) of the Pichia pastoris PAS8 gene, the PTS1 receptor (McCollum, D., E. Monosov, and S. Subramani. 1993. J. Cell Biol. 121:761-774)...
  2. Gould S, Kalish J, Morrell J, Bjorkman J, Urquhart A, Crane D. Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor. J Cell Biol. 1996;135:85-95 pubmed
    Import of newly synthesized PTS1 proteins into the peroxisome requires the PTS1 receptor (Pex5p), a predominantly cytoplasmic protein that cycles between the cytoplasm and peroxisome...
  3. Dodt G, Gould S. Multiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS1 receptor: evidence that PTS1 protein import is mediated by a cycling receptor. J Cell Biol. 1996;135:1763-74 pubmed
    ..In addition, we found that the PTS1 receptor was extremely unstable in the peroxin-deficient CG1, CG4, and CG8 cells...
  4. Fransen M, Terlecky S, Subramani S. Identification of a human PTS1 receptor docking protein directly required for peroxisomal protein import. Proc Natl Acad Sci U S A. 1998;95:8087-92 pubmed
    ..Functional analysis revealed that this 57-kDa molecule bound the PTS1 receptor, Pex5p, in ligand blots, suggesting it is a docking site on the peroxisomal membrane...
  5. Will G, Soukupova M, Hong X, Erdmann K, Kiel J, Dodt G, et al. Identification and characterization of the human orthologue of yeast Pex14p. Mol Cell Biol. 1999;19:2265-77 pubmed
    ..The role of HsPex14p in mammalian peroxisome biogenesis makes HsPEX14 a candidate PBD gene for being responsible for an unrecognized complementation group of human peroxisome biogenesis disorders. ..
  6. Gatto G, Geisbrecht B, Gould S, Berg J. Peroxisomal targeting signal-1 recognition by the TPR domains of human PEX5. Nat Struct Biol. 2000;7:1091-5 pubmed
    ..The PTS1 sequence closely approximates Ser-Lys-Leu-COO-. PEX5, the receptor for PTS1, interacts with the signal via a series of tetratricopeptide repeats (TPRs) within its C-..
  7. Huber P, Birdsey G, Lumb M, Prowse D, Perkins T, Knight D, et al. Peroxisomal import of human alanine:glyoxylate aminotransferase requires ancillary targeting information remote from its C terminus. J Biol Chem. 2005;280:27111-20 pubmed
    ..This interaction might reshape the tetratricopeptide repeat domain allosterically, enabling it to accept KKL as a functional PTS1. ..
  8. Carvalho A, Costa Rodrigues J, Correia I, Costa Pessoa J, Faria T, Martins C, et al. The N-terminal half of the peroxisomal cycling receptor Pex5p is a natively unfolded domain. J Mol Biol. 2006;356:864-75 pubmed
    Targeting of most newly synthesised peroxisomal matrix proteins to the organelle requires Pex5p, the so-called PTS1 receptor. According to current models of peroxisomal biogenesis, Pex5p interacts with these proteins in the cytosol, ..
  9. Stanley W, Pursiainen N, Garman E, Juffer A, Wilmanns M, Kursula P. A previously unobserved conformation for the human Pex5p receptor suggests roles for intrinsic flexibility and rigid domain motions in ligand binding. BMC Struct Biol. 2007;7:24 pubmed

More Information

Publications118 found, 100 shown here

  1. 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. ..
  2. Ghosh D, Berg J. A proteome-wide perspective on peroxisome targeting signal 1(PTS1)-Pex5p affinities. J Am Chem Soc. 2010;132:3973-9 pubmed publisher
    ..of a peroxisomal targeting signal-1 (PTS1), a short carboxy-terminal sequence specifically recognized by the PTS1 receptor Pex5p...
  3. 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...
  4. 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...
  5. Fransen M, Brees C, Baumgart E, Vanhooren J, Baes M, Mannaerts G, et al. Identification and characterization of the putative human peroxisomal C-terminal targeting signal import receptor. J Biol Chem. 1995;270:7731-6 pubmed
    ..All available evidence indicates that the PTS1-binding protein is part of the peroxisomal protein import machinery and most probably is the long sought after human PTS1 import receptor. ..
  6. 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
    ..We have isolated PXR1, a human homologue of the yeast P. pastoris PAS8 (peroxisome assembly) gene...
  7. Reuber B, Germain Lee E, Collins C, Morrell J, Ameritunga R, Moser H, et al. Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders. Nat Genet. 1997;17:445-8 pubmed
    ..of PEX1-deficient cells revealed severe defects in peroxisomal matrix protein import and destabilization of PEX5, the receptor for the type-1 peroxisomal targetting signal, even though peroxisomes were present in these cells and ..
  8. Otera H, Okumoto K, Tateishi K, Ikoma Y, Matsuda E, Nishimura M, et al. Peroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants. Mol Cell Biol. 1998;18:388-99 pubmed
    ..PEX5 cDNA, encoding the PTS1 receptor (PTS1R), was isolated from wild-type CHO-K1 cells...
  9. 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...
  10. Shimozawa N, Zhang Z, Suzuki Y, Imamura A, Tsukamoto T, Osumi T, et al. Functional heterogeneity of C-terminal peroxisome targeting signal 1 in PEX5-defective patients. Biochem Biophys Res Commun. 1999;262:504-8 pubmed
    ..The PTS1 receptor in 2-03 is functional for only the C-terminal -SKL sequence (acyl-CoA oxidase) and had little or no function ..
  11. Gatto G, Maynard E, Guerrerio A, Geisbrecht B, Gould S, Berg J. Correlating structure and affinity for PEX5:PTS1 complexes. Biochemistry. 2003;42:1660-6 pubmed
    ..The PTS1 is recognized by the tetratricopeptide repeat (TPR) domains of PEX5, a cytosolic receptor that cycles between the cytoplasm and the peroxisome...
  12. Miyata N, Fujiki Y. Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export. Mol Cell Biol. 2005;25:10822-32 pubmed
    ..matrix proteins are posttranslationally imported into peroxisomes with the peroxisome-targeting signal 1 receptor, Pex5. The longer isoform of Pex5, Pex5L, also transports Pex7-PTS2 protein complexes...
  13. Stanley W, Filipp F, Kursula P, Schüller N, Erdmann R, Schliebs W, et al. Recognition of a functional peroxisome type 1 target by the dynamic import receptor pex5p. Mol Cell. 2006;24:653-663 pubmed publisher
    ..The structure of the receptor/cargo complex demonstrates that the primary receptor-binding site of the cargo is structurally and topologically autonomous, enabling the cargo to retain its native structure and function. ..
  14. Grou C, Carvalho A, Pinto M, Wiese S, Piechura H, Meyer H, et al. Members of the E2D (UbcH5) family mediate the ubiquitination of the conserved cysteine of Pex5p, the peroxisomal import receptor. J Biol Chem. 2008;283:14190-7 pubmed publisher
    ..Here, we provide evidence suggesting that E2D1/2/3 (UbcH5a/b/c) are the mammalian functional counterparts of yeast/plant Pex4p. The mechanistic implications of these findings are discussed. ..
  15. Alencastre I, Rodrigues T, Grou C, Fransen M, Sa Miranda C, Azevedo J. Mapping the cargo protein membrane translocation step into the PEX5 cycling pathway. J Biol Chem. 2009;284:27243-51 pubmed publisher
    Newly synthesized peroxisomal matrix proteins are targeted to the organelle by PEX5, the peroxisomal cycling receptor...
  16. 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...
  17. Fodor K, Wolf J, Erdmann R, Schliebs W, Wilmanns M. Molecular requirements for peroxisomal targeting of alanine-glyoxylate aminotransferase as an essential determinant in primary hyperoxaluria type 1. PLoS Biol. 2012;10:e1001309 pubmed publisher
    ..We discuss how the knowledge of the molecular parameters for alanine-glyoxylate aminotransferase required for peroxisomal translocation could become useful for improved hyperoxaluria type 1 treatment. ..
  18. Freitas M, Francisco T, Rodrigues T, Lismont C, Domingues P, Pinto M, et al. The peroxisomal protein import machinery displays a preference for monomeric substrates. Open Biol. 2015;5:140236 pubmed publisher
    Peroxisomal matrix proteins are synthesized on cytosolic ribosomes and transported by the shuttling receptor PEX5 to the peroxisomal membrane docking/translocation machinery, where they are translocated into the organelle matrix...
  19. Wróblewska J, Cruz Zaragoza L, Yuan W, Schummer A, Chuartzman S, de Boer R, et al. Saccharomyces cerevisiae cells lacking Pex3 contain membrane vesicles that harbor a subset of peroxisomal membrane proteins. Biochim Biophys Acta Mol Cell Res. 2017;1864:1656-1667 pubmed publisher
    ..At the vesicles, Pex14 assembles with other peroxins (Pex13, Pex17, and Pex5) to form a complex with a composition similar to the PTS1 import pore in wild-type cells...
  20. Romano F, Blok N, Rapoport T. Peroxisome protein import recapitulated in Xenopus egg extracts. J Cell Biol. 2019;: pubmed publisher
    ..Most substrates contain a C-terminal Ser-Lys-Leu (SKL) sequence that is recognized by the receptor Pex5. Pex5 binds to peroxisomes via a docking complex containing Pex14, and recycles back into the cytosol following its ..
  21. 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-..
  22. Fan J, Li X, Issop L, Culty M, Papadopoulos V. ACBD2/ECI2-Mediated Peroxisome-Mitochondria Interactions in Leydig Cell Steroid Biosynthesis. Mol Endocrinol. 2016;30:763-82 pubmed publisher peroxisomal and mitochondrial matrix protein import complexes: peroxisomal import receptor peroxisomal biogenesis factor 5 (PEX5), and the mitochondrial import receptor subunit translocase of outer mitochondrial membrane 20 ..
  23. Sayols Baixeras S, Hernaéz A, Subirana I, Lluís Ganella C, Munoz D, Fito M, et al. DNA Methylation and High-Density Lipoprotein Functionality-Brief Report: The REGICOR Study (Registre Gironi del Cor). Arterioscler Thromb Vasc Biol. 2017;37:567-569 pubmed publisher
    ..We discovered 3 methylation sites located in HOXA3, PEX5, and PER3 related to cholesterol efflux capacity and 1 located in GABRR1 related to HDL ..
  24. Ahlemeyer B, Vogt J, Michel V, Hahn Kohlberger P, Baumgart Vogt E. Microporation is an efficient method for siRNA-induced knockdown of PEX5 in HepG2 cells: evaluation of the transfection efficiency, the PEX5 mRNA and protein levels and induction of peroxisomal deficiency. Histochem Cell Biol. 2014;142:577-91 pubmed publisher
    ..Therefore, several knockout models, e.g., the PEX5 knockout mouse, have been generated exhibiting a complete loss of peroxisomal function...
  25. Banerjee D, McDaniel P, Rymond B. Limited portability of G-patch domains in regulators of the Prp43 RNA helicase required for pre-mRNA splicing and ribosomal RNA maturation in Saccharomyces cerevisiae. Genetics. 2015;200:135-47 pubmed publisher
    ..In Saccharomyces cerevisiae, the Spp382/Ntr1, Sqs1/Pfa1, and Pxr1/Gno1 proteins are implicated as cofactors necessary for Prp43 helicase activation during spliceosome dissociation (..
  26. Thoms S. Import of proteins into peroxisomes: piggybacking to a new home away from home. Open Biol. 2015;5: pubmed publisher
    ..There is evidence that binding by Pex5 in the cytosol can regulate their oligomerization state before import...
  27. Di Cara F, Rachubinski R, Simmonds A. Distinct Roles for Peroxisomal Targeting Signal Receptors Pex5 and Pex7 in Drosophila. Genetics. 2019;211:141-149 pubmed publisher
    ..b>Pex5 and Pex7 are receptors that recognize different peroxisomal targeting signals called PTS1 and PTS2, respectively, ..
  28. Amer M, Elhefnawi M, El Ahwany E, Awad A, Gawad N, Zada S, et al. Hsa-miR-195 targets PCMT1 in hepatocellular carcinoma that increases tumor life span. Tumour Biol. 2014;35:11301-9 pubmed publisher
    ..The validation steps were done at two different levels in silico and in vitro. FGF7, GHR, PCMT1, CITED2, PEX5, PEX13, NOVA1, AXIN2, and TSPYL2 were detected with high significant (P < 0.005)...
  29. Hojjat H, Jardim A. Identification of Leishmania donovani peroxin 14 residues required for binding the peroxin 5 receptor proteins. Biochem J. 2015;465:247-57 pubmed publisher
    ..Biochemical studies revealed that these point mutations did not alter either the secondary or quaternary structure of LdPEX14 and indicated that the latter residues were critical for stabilizing the LdPEX5-LdPEX14 interaction. ..
  30. Meinecke M, Bartsch P, Wagner R. Peroxisomal protein import pores. Biochim Biophys Acta. 2016;1863:821-7 pubmed publisher
    ..The identification of a water-filled pore that is mainly constituted by Pex5 and Pex14 led to the assumption that proteins are translocated through a large, probably transient, protein-..
  31. Schwerter D, Grimm I, Girzalsky W, Erdmann R. Receptor recognition by the peroxisomal AAA complex depends on the presence of the ubiquitin moiety and is mediated by Pex1p. J Biol Chem. 2018;293:15458-15470 pubmed publisher
    ..This fusion protein displayed biological activity because it is able to functionally complement a PEX5-deletion in Saccharomyces cerevisiae...
  32. Rodrigues T, Grou C, Azevedo J. Revisiting the intraperoxisomal pathway of mammalian PEX7. Sci Rep. 2015;5:11806 pubmed publisher
    ..proteins containing a cleavable type 2 targeting signal (PTS2) are transported to the peroxisome by a cytosolic PEX5-PEX7 complex...
  33. Peeters A, Shinde A, Dirkx R, Smet J, De Bock K, Espeel M, et al. Mitochondria in peroxisome-deficient hepatocytes exhibit impaired respiration, depleted DNA, and PGC-1α independent proliferation. Biochim Biophys Acta. 2015;1853:285-98 pubmed publisher
    ..Here we investigated the molecular impairments of the dysfunctional mitochondria in hepatocyte selective Pex5 knockout mice...
  34. Skoulding N, Chowdhary G, Deus M, Baker A, Reumann S, Warriner S. Experimental validation of plant peroxisomal targeting prediction algorithms by systematic comparison of in vivo import efficiency and in vitro PTS1 binding affinity. J Mol Biol. 2015;427:1085-101 pubmed publisher
    ..In vitro binding to the PEX5 receptor could discriminate among strong targeting signals while in vivo targeting assays were more sensitive, ..
  35. Esmaeili M, Ghaedi K, Shoaraye Nejati A, Nematollahi M, Shiralyian H, Nasr Esfahani M. Pioglitazone significantly prevented decreased rate of neural differentiation of mouse embryonic stem cells which was reduced by Pex11β knock-down. Neuroscience. 2016;312:35-47 pubmed publisher
    ..Additionally, mRNA levels of other peroxisome-related genes such as PMP70, Pex11α, Catalase, Pex19 and Pex5 were also significantly reduced by Pex11β knock-down (p<0.05)...
  36. 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. ..
  37. 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
  38. Ramón N, Bartel B. Interdependence of the peroxisome-targeting receptors in Arabidopsis thaliana: PEX7 facilitates PEX5 accumulation and import of PTS1 cargo into peroxisomes. Mol Biol Cell. 2010;21:1263-71 pubmed publisher
    ..PEX5 is the PTS1 receptor; PEX7 is the PTS2 receptor...
  39. Pronicka E, Piekutowska Abramczuk D, Ciara E, Trubicka J, Rokicki D, Karkucinska Wieckowska A, et al. New perspective in diagnostics of mitochondrial disorders: two years' experience with whole-exome sequencing at a national paediatric centre. J Transl Med. 2016;14:174 pubmed publisher 18 non-MD related genes (ADAR, CACNA1A, CDKL5, CLN3, CPS1, DMD, DYSF, GBE1, GFAP, HSD17B4, MECP2, MYBPC3, PEX5, PGAP2, PIGN, PRF1, SBDS, SCN2A) were found...
  40. Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, et al. The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis. J Biol Chem. 1999;274:12593-604 pubmed
    ..Neither of these two mutant forms of Pex14p restored peroxisome biogenesis in ZP110 and ZP161, thereby demonstrating PEX14 to be responsible for peroxisome deficiency in CG14. ..
  41. Okumoto K, Noda H, Fujiki Y. Distinct modes of ubiquitination of peroxisome-targeting signal type 1 (PTS1) receptor Pex5p regulate PTS1 protein import. J Biol Chem. 2014;289:14089-108 pubmed publisher
    ..RING peroxins are required for both modes of Pex5p ubiquitination, thus playing a pivotal role in Pex5p shuttling. ..
  42. Dias A, Francisco T, Rodrigues T, Grou C, Azevedo J. The first minutes in the life of a peroxisomal matrix protein. Biochim Biophys Acta. 2016;1863:814-20 pubmed publisher
    ..In support of this idea, recent data suggest that PEX5, the shuttling receptor for peroxisomal matrix proteins, is also a chaperone/holdase, binding newly synthesized ..
  43. Styles N, Shonsey E, Falany J, Guidry A, Barnes S, Falany C. Carboxy-terminal mutations of bile acid CoA:N-acyltransferase alter activity and substrate specificity. J Lipid Res. 2016;57:1133-43 pubmed publisher
    ..BAAT-S immunoprecipitated when incubated with peroxisomal biogenesis factor 5 (Pex5) and rabbit anti-Pex5 antibodies; however, deleting the final 12 AAs prevented ..
  44. Mbekeani A, Stanley W, Kalel V, Dahan N, Zalckvar E, Sheiner L, et al. Functional Analyses of a Putative, Membrane-Bound, Peroxisomal Protein Import Mechanism from the Apicomplexan Protozoan Toxoplasma gondii. Genes (Basel). 2018;9: pubmed publisher
    ..proteins harbouring a peroxisomal-targeting signal 1 (PTS1) interact in the cytosol with the shuttling receptor Pex5 and are then imported into the peroxisome via the membrane-bound protein complex formed by Pex13 and Pex14...
  45. Duker A, Niiler T, Eldridge G, Brereton N, Braverman N, Bober M. Growth charts for individuals with rhizomelic chondrodysplasia punctata. Am J Med Genet A. 2017;173:108-113 pubmed publisher
    ..RCDP type 1 with PEX7, RCDP type 2 with GNPAT, RCDP type 3 with AGPS, RCDP type 4 with FAR1, and RCDP type 5 with PEX5. Among other medical/developmental issues, plasmalogen deficiency has a direct effect on bone growth and results in ..
  46. Mesa Torres N, Tomić N, Albert A, Salido E, Pey A. Molecular recognition of PTS-1 cargo proteins by Pex5p: implications for protein mistargeting in primary hyperoxaluria. Biomolecules. 2015;5:121-41 pubmed publisher
  47. Suaste Olmos F, Zirión Martínez C, Takano Rojas H, Peraza Reyes L. Meiotic development initiation in the fungus Podospora anserina requires the peroxisome receptor export machinery. Biochim Biophys Acta Mol Cell Res. 2018;1865:572-586 pubmed publisher
    ..However, this defect is not seen in the absence of the receptors PEX5 and PEX7, or of the docking peroxins PEX14 and PEX14/17...
  48. Walton P, Brees C, Lismont C, Apanasets O, Fransen M. The peroxisomal import receptor PEX5 functions as a stress sensor, retaining catalase in the cytosol in times of oxidative stress. Biochim Biophys Acta Mol Cell Res. 2017;1864:1833-1843 pubmed publisher
    ..By employing this approach, we confirm and extend previous observations that Cys-11 of human PEX5, the shuttling import receptor for peroxisomal matrix proteins containing a C-terminal peroxisomal targeting signal ..
  49. Francisco T, Rodrigues T, Freitas M, Grou C, Carvalho A, Sa Miranda C, et al. A cargo-centered perspective on the PEX5 receptor-mediated peroxisomal protein import pathway. J Biol Chem. 2013;288:29151-9 pubmed publisher
    ..matrix proteins are synthesized on cytosolic ribosomes and post-translationally targeted to the organelle by PEX5, the peroxisomal shuttling receptor...
  50. Kleinecke S, Richert S, de Hoz L, Brügger B, Kungl T, Asadollahi E, et al. Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy. elife. 2017;6: pubmed publisher
    ..Surprisingly, nerves of Schwann cell-specific Pex5 mutant mice were unaltered regarding axon numbers, axonal calibers, and myelin sheath thickness by electron ..
  51. 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 ..
  52. 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...
  53. Montilla Martinez M, Beck S, Klümper J, Meinecke M, Schliebs W, Wagner R, et al. Distinct Pores for Peroxisomal Import of PTS1 and PTS2 Proteins. Cell Rep. 2015;13:2126-34 pubmed publisher
    ..A pore consisting of the PTS1 receptor Pex5 and the docking protein Pex14 imports PTS1 proteins...
  54. Eun S, Lee J, Nam I, Liu Z, So H, Choe S, et al. PEX5 regulates autophagy via the mTORC1-TFEB axis during starvation. Exp Mol Med. 2018;50:4 pubmed publisher
    Defects in the PEX5 gene impair the import of peroxisomal matrix proteins, leading to nonfunctional peroxisomes and other associated pathological defects such as Zellweger syndrome...
  55. Nordgren M, Francisco T, Lismont C, Hennebel L, Brees C, Wang B, et al. Export-deficient monoubiquitinated PEX5 triggers peroxisome removal in SV40 large T antigen-transformed mouse embryonic fibroblasts. Autophagy. 2015;11:1326-40 pubmed publisher
    ..In this study, we show that PEX5 proteins fused to a bulky C-terminal tag trigger peroxisome degradation in SV40 large T antigen-transformed mouse ..
  56. Kao Y, Bartel B. Elevated growth temperature decreases levels of the PEX5 peroxisome-targeting signal receptor and ameliorates defects of Arabidopsis mutants with an impaired PEX4 ubiquitin-conjugating enzyme. BMC Plant Biol. 2015;15:224 pubmed publisher
    ..Most matrix proteins are delivered to peroxisomes by PEX5, a receptor that forms transient pores to escort proteins across the peroxisomal membrane...
  57. Kao Y, Fleming W, Ventura M, Bartel B. Genetic Interactions between PEROXIN12 and Other Peroxisome-Associated Ubiquitination Components. Plant Physiol. 2016;172:1643-1656 pubmed
    ..Peroxisomal matrix proteins carry peroxisome-targeting signals that are recognized by one of two receptors, PEX5 or PEX7, in the cytosol...
  58. Hojjat H, Jardim A. The Leishmania donovani peroxin 14 binding domain accommodates a high degeneracy in the pentapeptide motifs present on peroxin 5. Biochim Biophys Acta. 2015;1850:2203-12 pubmed publisher
  59. Lyman K, Han Y, Heuermann R, Cheng X, Kurz J, Lyman R, et al. Allostery between two binding sites in the ion channel subunit TRIP8b confers binding specificity to HCN channels. J Biol Chem. 2017;292:17718-17730 pubmed publisher
    ..For example, the TPR domains in the peroxisomal import receptor PEX5 enable binding to a range of type 1 peroxisomal targeting signal motifs...
  60. 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...
  61. Reumann S, Chowdhary G, Lingner T. Characterization, prediction and evolution of plant peroxisomal targeting signals type 1 (PTS1s). Biochim Biophys Acta. 2016;1863:790-803 pubmed publisher
    ..validated by in vivo subcellular targeting analyses and thermodynamic binding studies with the cytosolic receptor, PEX5. Accordingly, the algorithms allow the correct prediction of many novel peroxisome-targeted proteins from plant ..
  62. Cai M, Sun X, Wang W, Lian Z, Wu P, Han S, et al. Disruption of peroxisome function leads to metabolic stress, mTOR inhibition, and lethality in liver cancer cells. Cancer Lett. 2018;421:82-93 pubmed publisher
    ..Among the peroxins, PEX5 plays a transporter role, and PEX2, 10, and 12 are thought to form a complex that functions as an E3 ubiquitin ..
  63. Pedrosa A, Francisco T, Bicho D, Dias A, Barros Barbosa A, Hagmann V, et al. Peroxisomal monoubiquitinated PEX5 interacts with the AAA ATPases PEX1 and PEX6 and is unfolded during its dislocation into the cytosol. J Biol Chem. 2018;293:11553-11563 pubmed publisher
    ..Their role is to extract monoubiquitinated PEX5, the peroxisomal protein-shuttling receptor, from the peroxisomal membrane docking/translocation module (DTM), so ..
  64. Narayan V, Ly T, Pourkarimi E, Murillo A, Gartner A, Lamond A, et al. Deep Proteome Analysis Identifies Age-Related Processes in C. elegans. Cell Syst. 2016;3:144-159 pubmed publisher
    ..Among these are ∼30 peroxisomal proteins, including the PRX-5/PEX5 import protein...
  65. Luks L, Maier M, Sacchi S, Pollegioni L, Dietrich D. Understanding renal nuclear protein accumulation: an in vitro approach to explain an in vivo phenomenon. Arch Toxicol. 2017;91:3599-3611 pubmed publisher
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    ..We infer from these findings that targeted disruption of mouse Pex13 would provide an appropriate model for the study of PEX13 dysfunction in humans. ..
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