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Gene Review

PEX26  -  peroxisomal biogenesis factor 26

Homo sapiens

Synonyms: FLJ20695, PBD7A, PBD7B, PEX26M1T, Peroxin-26, ...
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High impact information on PEX26

  • Epitope-tagged Pex6 and Pex1 are discernible as puncta in normal CHO-K1 cells, but not in PEX26-defective cells [1].
  • 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, a Chinese hamster ovary (CHO) mutant cell line [1].
  • Expression of PEX26 restores peroxisomal protein import in the fibroblasts of an individual with PBD of CG8 [1].
  • This individual possesses a homozygous, inactivating pathogenic point mutation, Arg98Trp, in Pex26 [1].
  • They proposed that PEX26 functions as the peroxisomal docking factor for the PEX1/PEX6 heterodimer [2].

Biological context of PEX26


Anatomical context of PEX26

  • We found PEX26-Mito localized to the mitochondria and directed all detectable PEX6 and a fraction of PEX1 to this extraperoxisomal location; yet PEX26-Mito retains the full ability to rescue peroxisome biogenesis in PEX26-deficient cells [2].

Associations of PEX26 with chemical compounds

  • Although the PEX19-binding site containing the TMD targeted to peroxisomes to some extent, the luminal site proved essential for correct targeting of the full-length protein, as it prevented PEX26 from mislocalization to mitochondria [5].

Other interactions of PEX26

  • Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation [4].
  • Pex19p interacts with multiple membrane peroxins, including other membrane biogenesis peroxins, Pex16p and Pex26p, involved in matrix protein import [6].
  • To test our observation that a peroxisomal location is not required for PEX26 function, we made a chimeric protein (PEX26-Mito) with PEX26 as its N-terminus and the targeting segment of a mitochondrial outer membrane protein (OMP25) at its C-terminus [2].


  1. The pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomes. Matsumoto, N., Tamura, S., Fujiki, Y. Nat. Cell Biol. (2003) [Pubmed]
  2. Alternative splicing suggests extended function of PEX26 in peroxisome biogenesis. Weller, S., Cajigas, I., Morrell, J., Obie, C., Steel, G., Gould, S.J., Valle, D. Am. J. Hum. Genet. (2005) [Pubmed]
  3. Dynamic and Functional Assembly of the AAA Peroxins, Pex1p and Pex6p, and Their Membrane Receptor Pex26p. Tamura, S., Yasutake, S., Matsumoto, N., Fujiki, Y. J. Biol. Chem. (2006) [Pubmed]
  4. Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation. Matsumoto, N., Tamura, S., Furuki, S., Miyata, N., Moser, A., Shimozawa, N., Moser, H.W., Suzuki, Y., Kondo, N., Fujiki, Y. Am. J. Hum. Genet. (2003) [Pubmed]
  5. Targeting of the tail-anchored peroxisomal membrane proteins PEX26 and PEX15 occurs through C-terminal PEX19-binding sites. Halbach, A., Landgraf, C., Lorenzen, S., Rosenkranz, K., Volkmer-Engert, R., Erdmann, R., Rottensteiner, H. J. Cell. Sci. (2006) [Pubmed]
  6. In vitro transport of membrane proteins to peroxisomes by shuttling receptor Pex19p. Matsuzono, Y., Fujiki, Y. J. Biol. Chem. (2006) [Pubmed]
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