Peroxin 5 (Pex5) and Peroxin 7 (Pex7), are receptors for proteins that contain the peroxisome targeting signal 1 (PTS1) and 2 (PTS2), respectively, and shuttle these proteins from the cytosol to the peroxisome. Peroxin 5, also designated PTS1 receptor, is expressed as two isoforms, Pex5L and Pex5S. Pex5L transports PTS1 and Pex7-PTS2 cargo complexes to the initial Pex5 docking site, Pex14, while Pex5S transports only PTS1 cargoes. Pex5 and Pex7 also require either direct or indirect interaction with Peroxin 13 (Pex13) for proper import into peroxisomes. Mutations in the peroxin genes result in peroxisome biogenesis disorders (PBDs). Defects in the Pex5 gene are linked to Zellweger syndrome (cerebro-hapato-renal syndrome) of complementation group 2 (CG2), the most severe form of PBDs.
PCDP1; Peroxin 7; Peroxin-7; peroxisomal biogenesis factor 7; peroxisomal PTS2 receptor; Peroxisomal targeting signal 2 receptor; Peroxisome biogenesis factor 7; peroxisome targeting signal 2 receptor; PEX7; PEX7 protein; PTS2 receptor; PTS2R; RD