Target intelligence / Profile preview

Peroxisome assembly factor 2 (PEX6)

Target
PEX6
Molecular classification
Enzyme, AAA+ ATPase, Peroxin, Organelle biogenesis factor
01

Overview

Peroxisome assembly factor 2 (PEX6) is an AAA+ ATPase enzyme that forms a hexameric complex with PEX1; together, they are essential for peroxisome biogenesis by recycling the peroxisomal import receptor PEX5 from the peroxisomal membrane, enabling repeated rounds of matrix protein import. PEX6 is recruited to the peroxisome membrane via PEX26 and is crucial for maintaining peroxisomal function across eukaryotes. Mutations in PEX6 are a leading genetic cause of peroxisomal biogenesis disorders, such as Zellweger syndrome spectrum, indicating its critical role in cellular metabolism and development[1][2][3][4].

Other names
Peroxisomal biogenesis factor 6Peroxisomal ATPase PEX6Peroxin-6Peroxisomal-type ATPase 1Peroxisome assembly factor 2PXAAA1PAF-2PBD4APDB4Bperoxisomal AAA-type ATPase 1
02

Biological functions

Peroxisome biogenesisPeroxisomal protein importOrganelle maintenanceProtein unfolding and extractionOrganelle homeostasis
03

Disease associations

Peroxisomal biogenesis disordersZellweger spectrum disorderInfantile Refsum diseaseNeonatal adrenoleukodystrophyOther peroxisome-related metabolic diseases

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