Peroxisomal biogenesis factor 39 (PEX39)

Peroxisomal biogenesis factor 39 (PEX39) is a cytosolic peroxisomal biogenesis protein (peroxin) that facilitates the import of proteins containing a type 2 peroxisomal targeting signal (PTS2) by binding and stabilizing PEX7, the carrier for these cargo proteins. PEX39 carries a KPWE motif crucial for PEX7 interaction and operates alongside PEX13 to coordinate the sequential movement of cargo into the peroxisome. Proper levels of PEX39 are necessary for efficient protein import: both depletion and overexpression disrupt peroxisomal protein import and maturation. PEX39 dysfunction can cause severe peroxisomal disorders such as Zellweger Spectrum Disorders, and its discovery fills a longstanding gap in the molecular understanding of human peroxisome assembly.

Other names

C6orf226LOC441150uncharacterized protein C6orf226PEX39_HUMAN