Peroxisomal biogenesis factor 10 (PEX10)

Peroxisomal biogenesis factor 10 (PEX10) is an integral membrane protein of the peroxisome and a member of the peroxin protein family involved in peroxisome biogenesis[2][3]. PEX10 acts as an E3 ubiquitin-protein ligase and is a key component of the peroxisomal retrotranslocation channel for the receptor PEX5, collaborating with PEX2 and PEX12[3]. It facilitates the ubiquitination and export of PEX5 from peroxisomes, which is required for the import of matrix proteins—proteins that are needed within the peroxisome for various metabolic functions[3][5]. Loss of PEX10 function impairs peroxisome formation, matrix protein import, and proper peroxisome morphology, leading to severe developmental phenotypes and lethal peroxisomal biogenesis disorders in humans (including Zellweger syndrome) and plants (embryo lethality)[1][2][3]. Mutations in PEX10 are causally associated with peroxisome biogenesis disorders but no directly approved drugs target it, nor is it currently recognized as a therapeutic drug target or utilized as a clinical biomarker[3][5].

Other names

PEX10Peroxin 10