Peroxisomal biogenesis factor 13 (PEX13)

Peroxisomal biogenesis factor 13 (PEX13) is an integral peroxisomal membrane protein and a core component of the peroxisomal protein import machinery[3][2]. It interacts with the type 1 peroxisomal targeting signal receptor (PEX5) via an SH3 domain and forms the PEX13–PEX14 docking complex, which is essential for the import of peroxisomal matrix proteins[3]. PEX13 is also required for selective autophagy processes, such as mitophagy and virophagy, independently of general autophagy pathways[2][5]. Mutations in PEX13 cause peroxisome biogenesis disorders of the Zellweger syndrome spectrum, characterized by impaired peroxisomal protein import, leading to multi-organ, especially neurological, abnormalities and neonatal lethality[2][3][1][5]. The role of PEX13 is specific to peroxisome assembly and import, and it is not currently considered a direct therapeutic target or receptor in drug development[3][2][5].

Other names

Peroxisomal membrane protein PEX13Peroxin-13PBD11APBD11BZWSNALD