Peroxisomal biogenesis factor 19 (PEX19)

Peroxisomal biogenesis factor 19 (PEX19) is a cytosolic farnesylated protein required for the assembly, maintenance, and inheritance of peroxisomes in eukaryotic cells[3][4][1]. PEX19 functions primarily as a chaperone and import receptor for peroxisomal membrane proteins (PMPs), recognizing and binding newly synthesized PMPs in the cytosol, preventing their aggregation, and escorting them to the peroxisomal membrane via interaction with another factor, PEX3[1][2][4][5]. PEX19 is also directly involved in targeting and inserting PMPs into the peroxisomal membrane and plays roles in the division and inheritance of peroxisomes, particularly by interacting with PEX3 and PEX25 in yeast, or analogous pathways in mammals[1]. Mutations in the PEX19 gene can cause severe peroxisome biogenesis disorders (PBDs), notably Zellweger syndrome spectrum, a group of congenital disorders marked by multi-organ dysfunction, especially affecting the brain, liver, and skeletal system, typically resulting in early lethality[6][5].

Other names

D1S2223EHK33PBD12APMP1PMPIPXFPXMP1Peroxin-19Peroxisomal farnesylated protein33 kDa housekeeping proteinhousekeeping gene33kD