Gene Symbol: PEX13
Description: peroxisomal biogenesis factor 13
Alias: NALD, PBD11A, PBD11B, ZWS, peroxisome biogenesis factor 13, peroxin-13, peroxisomal membrane protein PEX13
Species: human
Products:     PEX13

Top Publications

  1. Albertini M, Rehling P, Erdmann R, Girzalsky W, Kiel J, Veenhuis M, et al. Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways. Cell. 1997;89:83-92 pubmed
    ..The data presented here are consistent with the idea of a common translocation machinery for both PTS-dependent protein import pathways in the peroxisomal membrane...
  2. 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
    ..These studies demonstrate that the ab-MF3-immunoreactive, 57-kDa peroxisomal membrane protein is Pex14p. Furthermore, this peroxin interacts with Pex5p and Pex13p(SH3) and is directly required for peroxisomal protein import...
  3. Bjorkman J, Stetten G, Moore C, Gould S, Crane D. Genomic structure of PEX13, a candidate peroxisome biogenesis disorder gene. Genomics. 1998;54:521-8 pubmed
    ..We have previously reported the identification of human PEX13, the gene encoding the docking factor for the PTS1 receptor, or PEX5 protein...
  4. 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
    ..We conclude that Pex13p functions as a docking factor for the predominantly cytoplasmic PTS1 receptor...
  5. Sacksteder K, Jones J, South S, Li X, Liu Y, Gould S. PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis. J Cell Biol. 2000;148:931-44 pubmed
    ..This hypothesis is supported by the observation that the loss of PEX19 results in degradation of PMPs and/or mislocalization of PMPs to the mitochondrion. ..
  6. Liu Y, Bjorkman J, Urquhart A, Wanders R, Crane D, Gould S. PEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders. Am J Hum Genet. 1999;65:621-34 pubmed
    ..b>PEX13 encodes a peroxisomal membrane protein with a cytoplasmically exposed SH3 domain, and we find that expression of ..
  7. Chai L, Zhang J, Lu K, Li H, Wu L, Wan H, et al. Identification of genomic regions associated with multi-silique trait in Brassica napus. BMC Genomics. 2019;20:304 pubmed publisher
    ..genomic regions responsible for the multi-silique trait using two pools of DNA from the near-isogenic lines (NILs) zws-ms (multi-silique) and zws-217 (single-silique)...
  8. Horna G, Lopez M, Guerra H, Sáenz Y, Ruiz J. Interplay between MexAB-OprM and MexEF-OprN in clinical isolates of Pseudomonas aeruginosa. Sci Rep. 2018;8:16463 pubmed publisher
    ..On the other hand, 33% of the isolates displayed RM in nalC, nalD or mexR, which were significantly associated with multidrug resistance (MDR), non-susceptibility to carbapenems, ..
  9. Hagmann V, Sommer S, Fabian P, Bierlmeier J, van Treel N, Mootz H, et al. Chemically monoubiquitinated PEX5 binds to the components of the peroxisomal docking and export machinery. Sci Rep. 2018;8:16014 pubmed publisher
    ..Here, we investigated the protein interactions of monoubiquitinated PEX5 with the docking proteins PEX13, PEX14 and the REM. "Click" chemistry was used to synthesise monoubiquitinated recombinant PEX5...

More Information


  1. Fernando H, Wiktorowicz J, Soman K, Kaphalia B, Khan M, Shakeel Ansari G. Liver proteomics in progressive alcoholic steatosis. Toxicol Appl Pharmacol. 2013;266:470-80 pubmed publisher
    Fatty liver is an early stage of alcoholic and nonalcoholic liver disease (ALD and NALD) that progresses to steatohepatitis and other irreversible conditions...
  2. Guder P, Lotz Havla A, Woidy M, Reiß D, Danecka M, Schatz U, et al. Isoform-specific domain organization determines conformation and function of the peroxisomal biogenesis factor PEX26. Biochim Biophys Acta Mol Cell Res. 2019;1866:518-531 pubmed publisher
    ..PEX26 and PEX26Δex5 displayed different patterns of interaction with PEX2-PEX10 or PEX13-PEX14 complexes, which relate to distinct pre-peroxisomes in the de novo synthesis pathway...
  3. 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
    ..shuttling receptor Pex5 and are then imported into the peroxisome via the membrane-bound protein complex formed by Pex13 and Pex14...
  4. 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)...
  5. Chen C, Chern S, Wu P, Chen S, Lai S, Chuang T, et al. Prenatal diagnosis of a 3.2-Mb 2p16.1-p15 duplication associated with familial intellectual disability. Taiwan J Obstet Gynecol. 2018;57:578-582 pubmed publisher
    ..encompassing eight Online Mendelian Inheritance in Man (OMIM) genes of VRK2, FANCL, BCL11A, PAPOLG, REL, PUS10, PEX13 and USP34 in the fetus and the two women. Prenatal ultrasound findings were unremarkable...
  6. Oruqaj G, Karnati S, Vijayan V, Kotarkonda L, Boateng E, Zhang W, et al. Compromised peroxisomes in idiopathic pulmonary fibrosis, a vicious cycle inducing a higher fibrotic response via TGF-β signaling. Proc Natl Acad Sci U S A. 2015;112:E2048-57 pubmed publisher
    ..Direct down-regulation of PEX13 by RNAi induced the activation of Smad-dependent TGF-β signaling accompanied by increased ROS production and ..
  7. Gao Y, Yang L, Chen J, Li Y, Jiang P, Zhang J, et al. Nitro and oxy-PAHs bounded in PM2.5 and PM1.0 under different weather conditions at Mount Tai in Eastern China: Sources, long-distance transport, and cancer risk assessment. Sci Total Environ. 2018;622-623:1400-1407 pubmed publisher
    ..22pg/m3). A similar trend was also reflected in measurements of PM1.0. 9-FO, 9,10-ANQ, and 1-NALD were the most abundant oxy-PAHs in both PM2.5 and PM1.0 at Mount Tai...
  8. Ajmal M, Yaccha M, Malik M, Rabbani M, Ahmad I, Isalm N, et al. Prevalence of nonalcoholic fatty liver disease (NAFLD) in patients of cardiovascular diseases and its association with hs-CRP and TNF-α. Indian Heart J. 2014;66:574-9 pubmed publisher
    ..Our data indicates that NALD is highly prevalent in patients of cardiovascular disease (69...
  9. Suresh M, Nithya N, Jayasree P, Vimal K, Manish Kumar P. Mutational analyses of regulatory genes, mexR, nalC, nalD and mexZ of mexAB-oprM and mexXY operons, in efflux pump hyperexpressing multidrug-resistant clinical isolates of Pseudomonas aeruginosa. World J Microbiol Biotechnol. 2018;34:83 pubmed publisher with membrane-bound efflux pumps, MexAB-OprM and MexXY and their respective regulatory genes mexR, nalC, nalD and mexZ in multidrug resistant (MDR) Pseudomonas aeruginosa...
  10. Morvay P, Baes M, Van Veldhoven P. Differential activities of peroxisomes along the mouse intestinal epithelium. Cell Biochem Funct. 2017;35:144-155 pubmed publisher
    ..Other peroxisomal enzymes (PHYH, HACL1, and ACAA1), the ABCD2 and ABCD3 transporters, and peroxins PEX13 and PEX14, however, did not follow this pattern, displaying rather constant signals throughout the intestinal ..
  11. Yuan W, Veenhuis M, van der Klei I. The birth of yeast peroxisomes. Biochim Biophys Acta. 2016;1863:902-10 pubmed publisher
    ..This is especially evident for pex3 and pex19 mutants, where the localization of receptor docking proteins (Pex13, Pex14) resulted in the identification of peroxisomal membrane remnants, which do not contain other peroxisomal ..
  12. Qian G, Fan W, Ahlemeyer B, Karnati S, Baumgart Vogt E. Peroxisomes in Different Skeletal Cell Types during Intramembranous and Endochondral Ossification and Their Regulation during Osteoblast Differentiation by Distinct Peroxisome Proliferator-Activated Receptors. PLoS ONE. 2015;10:e0143439 pubmed publisher
    ..GW0742 activated the PPRE-mediated luciferase expression and up-regulated peroxisomal gene transcription (Pex11, Pex13, Pex14, Acox1 and Cat), whereas the PPARß antagonist GSK0660 led to repression of the PPRE and a decrease of ..
  13. Rahim R, Chen M, Nourse C, Meedeniya A, Crane D. Mitochondrial changes and oxidative stress in a mouse model of Zellweger syndrome neuropathogenesis. Neuroscience. 2016;334:201-213 pubmed publisher
    ..Using a mouse model of ZS with brain-restricted deficiency of the peroxisome biogenesis protein PEX13, we demonstrated an expanded and morphologically modified brain mitochondrial population...
  14. Hu F, Chen K, Xu H, Gu H. Design and preparation of bi-functionalized short-chain modified zwitterionic nanoparticles. Acta Biomater. 2018;72:239-247 pubmed publisher
    ..In this paper, the carboxyl end group of short-chain zwitterion-coated silica nanoparticles (SiO2-ZWS) was found to be difficult to functionalize via a conventional EDC/NHS strategy due to its rapid hydrolysis side-..
  15. Agrawal G, Fassas S, Xia Z, Subramani S. Distinct requirements for intra-ER sorting and budding of peroxisomal membrane proteins from the ER. J Cell Biol. 2016;212:335-48 pubmed publisher
    ..Although the docking subcomplex proteins (Pex13, Pex14, and Pex17) also required Pex19 for budding from the ER, they sorted to the pER independently of Pex3 and ..
  16. Xu Z, Asahchop E, Branton W, Gelman B, Power C, Hobman T. MicroRNAs upregulated during HIV infection target peroxisome biogenesis factors: Implications for virus biology, disease mechanisms and neuropathology. PLoS Pathog. 2017;13:e1006360 pubmed publisher
    ..miR-93-3p and miR-381-3p), are predicted to target peroxisome biogenesis factors (PEX2, PEX7, PEX11B and PEX13)...
  17. Sugiura A, Mattie S, Prudent J, McBride H. Newly born peroxisomes are a hybrid of mitochondrial and ER-derived pre-peroxisomes. Nature. 2017;542:251-254 pubmed publisher
    ..proteins are imported into mitochondria in mammalian cells in the absence of peroxisomes, including Pex3, Pex12, Pex13, Pex14, Pex26, PMP34 and ALDP...
  18. Pan Y, Xu Y, Wang Z, Fang Y, Shen J. Overexpression of MexAB-OprM efflux pump in carbapenem-resistant Pseudomonas aeruginosa. Arch Microbiol. 2016;198:565-71 pubmed publisher
    ..resistant efflux pumps with high-level expression, which is controlled by regulatory genes mexR, nalC, and nalD. This study investigated the role of efflux pump MexAB-OprM in 75 strains of carbapenem-resistant P...
  19. 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
    ..One such process is the meiotic development of Podospora anserina. In this fungus, absence of the docking peroxin PEX13, the RING-finger complex peroxins, or the PTS2 co-receptor PEX20 blocks sexual development before meiocyte ..
  20. Mimouni Bloch A, Yeshaya J, Kahana S, Maya I, Basel Vanagaite L. A de-novo interstitial microduplication involving 2p16.1-p15 and mirroring 2p16.1-p15 microdeletion syndrome: Clinical and molecular analysis. Eur J Paediatr Neurol. 2015;19:711-5 pubmed publisher
    ..The involvement of genes in this location including BCL11A, USP34 and PEX13, affecting fundamental developmental processes both within and outside the nervous system may explain the clinical ..
  21. Pan R, Satkovich J, Hu J. E3 ubiquitin ligase SP1 regulates peroxisome biogenesis in Arabidopsis. Proc Natl Acad Sci U S A. 2016;113:E7307-E7316 pubmed
    ..SP1 interacts physically with the two components of the peroxisome protein docking complex PEX13-PEX14 and the (RING)-finger peroxin PEX2...
  22. Knoops K, de Boer R, Kram A, van der Klei I. Yeast pex1 cells contain peroxisomal ghosts that import matrix proteins upon reintroduction of Pex1. J Cell Biol. 2015;211:955-62 pubmed publisher
    ..peroxisomal matrix proteins but are the sole sites of the major peroxisomal membrane proteins Pex2, Pex10, Pex11, Pex13, and Pex14...
  23. Chen W, Wang D, Zhou W, Sang H, Liu X, Ge Z, et al. Novobiocin binding to NalD induces the expression of the MexAB-OprM pump in Pseudomonas aeruginosa. Mol Microbiol. 2016;100:749-58 pubmed publisher
    b>NalD was reported to be the secondary repressor of the MexAB-OprM multidrug efflux pump, the major system contributing to intrinsic multidrug resistance in Pseudomonas aeruginosa...
  24. Jorth P, McLean K, Ratjen A, Secor P, Bautista G, Ravishankar S, et al. Evolved Aztreonam Resistance Is Multifactorial and Can Produce Hypervirulence in Pseudomonas aeruginosa. MBio. 2017;8: pubmed publisher
    ..strains carrying evolved mutations which affected negative regulators of mexAB-oprM (mexR and nalD) demonstrated enhanced virulence in a murine pneumonia infection model...
  25. Fransen M, Brees C, Ghys K, Amery L, Mannaerts G, Ladant D, et al. Analysis of mammalian peroxin interactions using a non-transcription-based bacterial two-hybrid assay. Mol Cell Proteomics. 2002;1:243-52 pubmed
  26. Hashimoto K, Kato Z, Nagase T, Shimozawa N, Kuwata K, Omoya K, et al. Molecular mechanism of a temperature-sensitive phenotype in peroxisomal biogenesis disorder. Pediatr Res. 2005;58:263-9 pubmed
    ..of a NALD patient with a marked deterioration by a fever revealed a mutation (Ile326Thr) within a SH3 domain of PEX13 protein (Pex13p), showing a temperature-sensitive (TS) phenotype in peroxisomal biogenesis...
  27. Girzalsky W, Rehling P, Stein K, Kipper J, Blank L, Kunau W, et al. Involvement of Pex13p in Pex14p localization and peroxisomal targeting signal 2-dependent protein import into peroxisomes. J Cell Biol. 1999;144:1151-62 pubmed
    ..We also found Pex13p to be required for peroxisomal association of Pex14p, yet the SH3 domain of Pex13p may not provide the only binding site for Pex14p at the peroxisomal membrane...
  28. Dias A, Rodrigues T, Pedrosa A, Barros Barbosa A, Francisco T, Azevedo J. The peroxisomal matrix protein translocon is a large cavity-forming protein assembly into which PEX5 protein enters to release its cargo. J Biol Chem. 2017;292:15287-15300 pubmed publisher
    ..Collectively, these results suggest that the DTM is best described as a large cavity-forming protein assembly into which cytosolic PEX5 can enter to release its cargo. ..
  29. Krause C, Rosewich H, Woehler A, Gartner J. Functional analysis of PEX13 mutation in a Zellweger syndrome spectrum patient reveals novel homooligomerization of PEX13 and its role in human peroxisome biogenesis. Hum Mol Genet. 2013;22:3844-57 pubmed publisher
    ..Previously, we identified the W313G mutation located within the SH3 domain of the peroxisomal protein, PEX13. As this tryptophan residue is highly conserved in almost all known SH3 proteins, we investigated the pathogenic ..
  30. Schueller N, Holton S, Fodor K, Milewski M, Konarev P, Stanley W, et al. The peroxisomal receptor Pex19p forms a helical mPTS recognition domain. EMBO J. 2010;29:2491-500 pubmed publisher
  31. Otera H, Setoguchi K, Hamasaki M, Kumashiro T, Shimizu N, Fujiki Y. 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. Mol Cell Biol. 2002;22:1639-55 pubmed
    ..Thus, Pex14p and Pex13p very likely form mutually and temporally distinct subcomplexes involved in peroxisomal matrix protein import...
  32. Al Dirbashi O, Shaheen R, al Sayed M, Al Dosari M, Makhseed N, Abu Safieh L, et al. Zellweger syndrome caused by PEX13 deficiency: report of two novel mutations. Am J Med Genet A. 2009;149A:1219-23 pubmed publisher
    ..Mutations in PEX13, which encodes peroxisomal membrane protein PEX13, are among the least common causes of peroxisomal biogenesis disorders with only three mutations ..
  33. Fransen M, Vastiau I, Brees C, Brys V, Mannaerts G, Van Veldhoven P. Potential role for Pex19p in assembly of PTS-receptor docking complexes. J Biol Chem. 2004;279:12615-24 pubmed
    ..Importantly, we also found that amino acid substitutions resulting in a strongly reduced binding affinity for Pex13p affect the peroxisomal localization of Pex14p. ..
  34. Nguyen T, Bjorkman J, Paton B, Crane D. Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance. J Cell Sci. 2006;119:636-45 pubmed
    ..Similar effects were observed for both cultured embryonic fibroblasts and brain neurons from a PEX13-null mouse with a Zellweger-syndrome-like phenotype, and a less-pronounced effect was observed for fibroblasts ..
  35. Nenicu A, Lüers G, Kovacs W, David M, Zimmer A, Bergmann M, et al. Peroxisomes in human and mouse testis: differential expression of peroxisomal proteins in germ cells and distinct somatic cell types of the testis. Biol Reprod. 2007;77:1060-72 pubmed
    ..Our results suggest a cell type-specific metabolic function of peroxisomes in the testis and point to an important role for peroxisomes in spermiogenesis and in the lipid metabolism of Sertoli cells. ..
  36. Rahim R, St John J, Crane D, Meedeniya A. Impaired neurogenesis and associated gliosis in mouse brain with PEX13 deficiency. Mol Cell Neurosci. 2018;88:16-32 pubmed publisher
    ..Here we used mice with brain-restricted inactivation of the peroxisome biogenesis gene PEX13 to model the pathophysiological features of ZS, and determine the impact of peroxisome dysfunction on neurogenesis ..
  37. Toyama R, Mukai S, Itagaki A, Tamura S, Shimozawa N, Suzuki Y, et al. Isolation, characterization and mutation analysis of PEX13-defective Chinese hamster ovary cell mutants. Hum Mol Genet. 1999;8:1673-81 pubmed
    ..Expression of human PEX13 cDNA restored peroxisome assembly in ZP128 and ZP150...
  38. Shimozawa N, Suzuki Y, Zhang Z, Imamura A, Toyama R, Mukai S, et al. Nonsense and temperature-sensitive mutations in PEX13 are the cause of complementation group H of peroxisome biogenesis disorders. Hum Mol Genet. 1999;8:1077-83 pubmed
    ..In addition, we detected mutations on the human PEX13 cDNA in two patients of group H...
  39. Fransen M, Wylin T, Brees C, Mannaerts G, Van Veldhoven P. Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences. Mol Cell Biol. 2001;21:4413-24 pubmed
  40. Jones J, Morrell J, Gould S. Multiple distinct targeting signals in integral peroxisomal membrane proteins. J Cell Biol. 2001;153:1141-50 pubmed
    ..We also show that another integral PMP, the peroxin PEX13, also contains two independent sets of peroxisomal targeting information...
  41. 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
    b>Pex13 encodes an SH3-containing peroxisomal membrane protein required for the import of proteins into peroxisomes...
  42. Demetris A, Specht S, Nozaki I, Lunz J, Stolz D, Murase N, et al. Small proline-rich proteins (SPRR) function as SH3 domain ligands, increase resistance to injury and are associated with epithelial-mesenchymal transition (EMT) in cholangiocytes. J Hepatol. 2008;48:276-88 pubmed
    ..These observations suggest that SPRR2A-related BEC barrier modifications represent a novel, but widely utilized and evolutionarily conserved, response to stress that is worthy of further study. ..
  43. Bavia L, Cogliati B, Dettoni J, Ferreira Alves V, Isaac L. The complement component C5 promotes liver steatosis and inflammation in murine non-alcoholic liver disease model. Immunol Lett. 2016;177:53-61 pubmed publisher
    Non-Alcoholic Fatty Liver Disease (NALD) is considering a hepatic manifestation of metabolic syndrome. Although the pathogenesis of NALD is not completely understood, insulin resistance and inflammatory cytokines are implicated...
  44. 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...
  45. Baarine M, Andreoletti P, Athias A, Nury T, Zarrouk A, Ragot K, et al. Evidence of oxidative stress in very long chain fatty acid--treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins. Neuroscience. 2012;213:1-18 pubmed publisher
    X-linked adrenoleukodystrophy (X-ALD) and pseudo neonatal adrenoleukodystrophy (P-NALD) are neurodegenerative demyelinating diseases resulting from the functional loss of the peroxisomal ATP-binding cassette transporter D (ABCD1) and from ..
  46. Lee M, Sumpter R, Zou Z, Sirasanagandla S, Wei Y, Mishra P, et al. Peroxisomal protein PEX13 functions in selective autophagy. EMBO Rep. 2017;18:48-60 pubmed publisher
    b>PEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis...
  47. Braz V, Furlan J, Fernandes A, Stehling E. Mutations in NalC induce MexAB-OprM overexpression resulting in high level of aztreonam resistance in environmental isolates of Pseudomonas aeruginosa. FEMS Microbiol Lett. 2016;363: pubmed publisher
    ..efflux of various antibiotics, mostly when mutations accumulate in the transcriptional regulators MexR, NalC and NalD, thereby causing MexAB-OprM overexpression. In this work, a characterization of 50 P...
  48. Haenni M, Bour M, Châtre P, Madec J, Plesiat P, Jeannot K. Resistance of Animal Strains of Pseudomonas aeruginosa to Carbapenems. Front Microbiol. 2017;8:1847 pubmed publisher
    ..8; activation of two-component system ParRS), or MexAB-OprM (n = 12; alteration of regulator MexR, NalC ± NalD). Two efflux pumps were co-produced simultaneously in three mutants...