The pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomes

Naomi Matsumoto, Shigehiko Tamura, Yukio Fujiki

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155 Citations (Scopus)


Peroxisomes are ubiquitous organelles with a single membrane that contain over 50 different enzymes that catalyse various metabolic pathways, including β-oxidation and lipid synthesis1. Peroxisome biogenesis disorders (PBDs), such as Zellweger syndrome and neonatal adrenoleukodystrophy, are fatal genetic diseases that are autosomal recessive2,3 . Among the PBDs of the 12 complementation groups (CGs)4, 11 associated PEX genes have been isolated4-7 . Accordingly, only the PBD pathogenic gene for CG8 (also called CG-A) remains unidentified. Here we have isolated human PEX26 encoding a type II peroxisomal membrane protein of relative molecular mass 34,000 (Mr 34K) by using ZP167 cells, a Chinese hamster ovary (CHO) mutant cell line5,8. Expression of PEX26 restores peroxisomal protein import in the fibroblasts of an individual with PBD of CG8. This individual possesses a homozygous, inactivating pathogenic point mutation, Arg98Trp, in Pex26. Pex6 and Pex1 of the AAA ATPase family co-immunoprecipitate with Pex26. Epitopetagged Pex6 and Pex1 are discernible as puncta in normal CHO-K1 cells, but not in PEX26-defective cells. PEX26 expression in ZP167 cells re-establishes colocalization of Pex6 and Pex1 with Pex26, in a Pex6-dependent manner. Thus, Pex26 recruits Pex6-Pex1 complexes to peroxisomes.

Original languageEnglish
Pages (from-to)454-460
Number of pages7
JournalNature Cell Biology
Issue number5
Publication statusPublished - May 1 2003

All Science Journal Classification (ASJC) codes

  • Cell Biology


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