Peroxisome assembly protein 26 (PEX26)

Peroxisome assembly protein 26 (PEX26) is an integral membrane protein of the peroxisome that serves as a membrane anchor for the PEX1–PEX6 AAA ATPase complex, which is critical for peroxisomal matrix protein import and peroxisome biogenesis. PEX26 is required for docking PEX1 and PEX6 to the peroxisomal membrane, enabling the extraction of the PEX5 import receptor from the peroxisome following cargo delivery. It is involved in additional processes such as peroxisomal division, proliferation, and various aspects of lipid metabolism, including fatty acid β-oxidation and plasmalogen synthesis. Loss-of-function mutations in PEX26 cause a subset of peroxisome biogenesis disorders, including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease, which are characterized by impaired peroxisomal matrix protein import and broad metabolic dysfunction. Several alternatively spliced isoforms exist, some of which can partially complement PEX26 deficiency. PEX26 is not a direct therapeutic target but is a key node in peroxisomal biogenesis and disease, with its deficiency resulting in variably severe neurological and metabolic disorders.

Other names

Peroxin-26Peroxisomal biogenesis factor 26FLJ20695PBD7APBD7BPEX26M1TPex26pM1Tperoxisome biogenesis disorder complementation group 8 proteinperoxisome biogenesis disorder complementation group A protein

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