Gene Symbol: PEX26
Description: peroxisomal biogenesis factor 26
Alias: PBD7A, PBD7B, PEX26M1T, Pex26pM1T, peroxisome assembly protein 26, peroxin-26, peroxisome biogenesis disorder, complementation group 8, peroxisome biogenesis disorder, complementation group A, peroxisome biogenesis factor 26
Species: human
Products:     PEX26

Top Publications

  1. Matsumoto N, Tamura S, Fujiki Y. The pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomes. Nat Cell Biol. 2003;5:454-60 pubmed
    ..Here we have isolated human PEX26 encoding a type II peroxisomal membrane protein of relative molecular mass 34,000 (M(r) 34K) by using ZP167 cells, ..
  2. Halbach A, Landgraf C, Lorenzen S, Rosenkranz K, Volkmer Engert R, Erdmann R, et al. Targeting of the tail-anchored peroxisomal membrane proteins PEX26 and PEX15 occurs through C-terminal PEX19-binding sites. J Cell Sci. 2006;119:2508-17 pubmed
    ..Here we analyzed targeting of the human peroxisomal tail-anchored protein PEX26. Its C-terminal-targeting signal contains two binding sites for PEX19, the import receptor for several peroxisomal ..
  3. Khan H, Mew N, Kaufman S, Yazigi N, Fishbein T, Khan K. Unusual Cystic Fibrosis Transmembrane Conductance Regulator Mutations and Liver Disease: A Case Series and Review of the Literature. Transplant Proc. 2019;51:790-793 pubmed publisher
    ..A sweat test was negative and genetic testing revealed she was heterozygous for CFTR and PEX26 gene mutations...
  4. Tanaka A, Okumoto K, Tamura S, Abe Y, Hirsch Y, Deng L, et al. A newly identified mutation in the PEX26 gene is associated with a milder type of Zellweger spectrum disorder. Cold Spring Harb Mol Case Stud. 2018;: pubmed publisher
    ..153C>A (p.F51L), in the peroxisome biogenesis factor 26 gene (PEX26) in a 19-year old female who was referred for moderate to severe hearing loss...
  5. 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 monoubiquitinated and extracted from the peroxisomal membrane by the receptor export machinery (REM) comprising PEX26 and the AAA ATPases PEX1 and PEX6...
  6. 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
    Peroxisomal biogenesis factor PEX26 is a membrane anchor for the multi-subunit PEX1-PEX6 protein complex that controls ubiquitination and dislocation of PEX5 cargo receptors for peroxisomal matrix protein import...
  7. Wang X, Yik W, Zhang P, Lu W, Huang N, Kim B, et al. Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities. Stem Cell Res Ther. 2015;6:158 pubmed publisher
    ..Primary skin fibroblasts from seven PBD-ZSD patients with biallelic PEX1, PEX10, PEX12, or PEX26 mutations and three healthy donors were transduced with retroviral vectors expressing Yamanaka reprogramming ..
  8. 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
    ..imported into mitochondria in mammalian cells in the absence of peroxisomes, including Pex3, Pex12, Pex13, Pex14, Pex26, PMP34 and ALDP...
  9. Chen F, Lei T, Fu F, Li R, Zhang Y, Jing X, et al. [Application of chromosome microarray analysis for fetuses with multicystic dysplastic kidney]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016;33:752-757 pubmed
    ..2, 22q13.33, and 1p33. PEX26, FKBP6, TUBGCP6, ALG12, and CYP4A11 are likely the causative genes...

More Information


  1. Gonzalez K, Ratzel S, Burks K, Danan C, Wages J, Zolman B, et al. A pex1 missense mutation improves peroxisome function in a subset of Arabidopsis pex6 mutants without restoring PEX5 recycling. Proc Natl Acad Sci U S A. 2018;115:E3163-E3172 pubmed publisher
    ..peroxisome function in various pex6 alleles but worsened the physiological and molecular defects of a pex26 mutant, which is defective in the tether anchoring the PEX1-PEX6 hexamer to the peroxisome...
  2. 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
    ..potentially mediating receptor monoubiquitination (PEX4 and PEX22) and membrane dislocation (PEX1, PEX6 and PEX26) are indeed implicated in peroxisome matrix protein import in this fungus...
  3. Steinberg S, Chen L, Wei L, Moser A, Moser H, Cutting G, et al. The PEX Gene Screen: molecular diagnosis of peroxisome biogenesis disorders in the Zellweger syndrome spectrum. Mol Genet Metab. 2004;83:252-63 pubmed
    ..PCR amplification of genomic DNA and sequencing to screen 91 unclassified PBD-ZSS patients for mutations in PEX1, PEX26, PEX6, PEX12, PEX10, and PEX2...
  4. Nazarko T. Pexophagy is responsible for 65% of cases of peroxisome biogenesis disorders. Autophagy. 2017;13:991-994 pubmed publisher
    ..make up the peroxisomal AAA ATPase complex (AAA-complex) in yeast (Pex1, Pex6 and Pex15) or mammals (PEX1, PEX6, PEX26) are responsible for the downregulation of pexophagy...
  5. Yagita Y, Hiromasa T, Fujiki Y. Tail-anchored PEX26 targets peroxisomes via a PEX19-dependent and TRC40-independent class I pathway. J Cell Biol. 2013;200:651-66 pubmed publisher
    ..The present study shows that PEX19 formed a complex with the peroxisomal TA protein PEX26 in the cytosol and translocated it directly to peroxisomes by interacting with the peroxisomal membrane protein ..
  6. Matsumoto N, Tamura S, Furuki S, Miyata N, Moser A, Shimozawa N, et al. Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation. Am J Hum Genet. 2003;73:233-46 pubmed
    ..b>PEX26, encoding the 305-amino-acid membrane peroxin, has been shown to be deficient in CG8...
  7. Weller S, Cajigas I, Morrell J, Obie C, Steel G, Gould S, et al. Alternative splicing suggests extended function of PEX26 in peroxisome biogenesis. Am J Hum Genet. 2005;76:987-1007 pubmed
    Matsumoto and colleagues recently identified PEX26 as the gene responsible for complementation group 8 of the peroxisome biogenesis disorders and showed that it encodes an integral peroxisomal membrane protein with a single C-terminal ..
  8. Fransen M, Vastiau I, Brees C, Brys V, Mannaerts G, Van Veldhoven P. Analysis of human Pex19p's domain structure by pentapeptide scanning mutagenesis. J Mol Biol. 2005;346:1275-86 pubmed
    ..Finally, our observation that Pex19p contains two distinct binding sites for Pex3p suggests that the peroxin may bind PMPs in multiple places and for multiple purposes. ..
  9. Buentzel J, Vilardi F, Lotz Havla A, Gärtner J, Thoms S. Conserved targeting information in mammalian and fungal peroxisomal tail-anchored proteins. Sci Rep. 2015;5:17420 pubmed publisher
    ..Pex15 and PEX26, tail-anchored proteins in yeast and mammals, respectively, exert a similar cellular function in the recruitment ..
  10. Tamura S, Matsumoto N, Takeba R, Fujiki Y. AAA peroxins and their recruiter Pex26p modulate the interactions of peroxins involved in peroxisomal protein import. J Biol Chem. 2014;289:24336-46 pubmed publisher
    ..Taken together, in the peroxisomal protein import, AAA peroxins modulate the interaction between Pex26p and Pex14p on peroxisome membrane as well as Pex5p oligomer in the cytosol. ..
  11. Yik W, Steinberg S, Moser A, Moser H, Hacia J. Identification of novel mutations and sequence variation in the Zellweger syndrome spectrum of peroxisome biogenesis disorders. Hum Mutat. 2009;30:E467-80 pubmed publisher
    ..Mutations in the PEX1, PEX6, PEX10, PEX12, or PEX26 genes are found in approximately 90% of PBD-ZSS patients...
  12. Tamura S, Yasutake S, Matsumoto N, Fujiki Y. Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p. J Biol Chem. 2006;281:27693-704 pubmed
    ..b>PEX26 responsible for peroxisome biogenesis disorders of CG8 encodes Pex26p, the recruiter of Pex1p...
  13. Furuki S, Tamura S, Matsumoto N, Miyata N, Moser A, Moser H, et al. Mutations in the peroxin Pex26p responsible for peroxisome biogenesis disorders of complementation group 8 impair its stability, peroxisomal localization, and interaction with the Pex1p x Pex6p complex. J Biol Chem. 2006;281:1317-23 pubmed
    ..We recently isolated PEX26 as the pathogenic gene for PBD of CG8...
  14. 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
  15. Gonzalez K, Fleming W, Kao Y, Wright Z, Venkova S, Ventura M, et al. Disparate peroxisome-related defects in Arabidopsis pex6 and pex26 mutants link peroxisomal retrotranslocation and oil body utilization. Plant J. 2017;92:110-128 pubmed publisher
    ..After cargo delivery, a complex of the PEX1 and PEX6 ATPases and the PEX26 tail-anchored membrane protein removes ubiquitinated PEX5 from the peroxisomal membrane...