Peroxisomal biogenesis factor 3 (PEX3)

Peroxisomal biogenesis factor 3 (PEX3) is an integral peroxisomal membrane protein that plays a crucial role in the early steps of peroxisome formation and the import of membrane proteins into peroxisomes[1][2][3]. PEX3 acts as a docking receptor on the peroxisome membrane for cytosolic PEX19, which delivers and inserts newly synthesized peroxisomal membrane proteins. It is also essential for peroxisome biosynthesis and integrity, assembling membrane vesicles prior to matrix protein translocation[1][3]. In addition to its canonical role in membrane assembly, PEX3 is implicated in the regulation of pexophagy, a selective autophagy process that removes damaged or surplus peroxisomes through interactions with autophagy receptors such as Atg30[2]. Mutations in PEX3 result in peroxisome biogenesis disorders (PBDs), a group of genetic diseases—most notably in Zellweger spectrum disorders—characterized by severe defects in peroxisome function and metabolic abnormalities[3]. PEX3 is not a classical therapeutic target like a receptor or enzyme and, to date, no drugs specifically target PEX3. There are no validated biomarkers or known safety concerns directly associated with modulating PEX3, though its loss-of-function has severe embryonic or childhood disease consequences[1][3].

Other names

Peroxisomal biogenesis factor 3PEX3Peroxin-3Peroxisomal assembly protein PEX3PBD10APBD10BTRG18transformation-related protein 18