Peroxisomal targeting signal 1 receptor (PEX5)

Peroxisomal targeting signal 1 receptor (PEX5) is a cytosolic receptor essential for peroxisomal matrix protein import. PEX5 recognizes and binds proteins carrying a C-terminal peroxisomal targeting signal 1 (PTS1), typically a Ser-Lys-Leu (SKL)-type tripeptide, and transports them from the cytosol into peroxisomes by interacting with the peroxisomal membrane docking complexes, primarily PEX13 and PEX14[1][3][4]. A longer isoform (PEX5L) also facilitates the import of peroxisomal PTS2-protein complexes via interaction with another peroxin, PEX7[3]. PEX5 plays a critical role in peroxisome biogenesis and function; pathogenic variants in PEX5 cause inherited peroxisome biogenesis disorders, notably Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease[1][3]. Defective PEX5 activity results in disruption of peroxisomal protein import and multisystem metabolic disease[1][3].

Other names

PXR1PBD2APBD2BPTS1RRCDP5PTS1-BPPeroxin-5Peroxisomal import receptor 5Peroxisome receptor 1Peroxisomal biogenesis factor 5Peroxisomal C-terminal targeting signal import receptor