InterPro domain: IPR044536

Peroxisomal proteins catalyse metabolic reactions. The import of proteins from the cytosol into the peroxisomes matrix depends on more than a dozen peroxin (PEX) proteins, among which PEX5 and PEX7 serve as receptors that shuttle proteins bearing one of two peroxisome-targeting signals (PTSs) into the organelle. PEX5 is the PTS1 receptor, while PEX7 is the PTS2 receptor. In plants, PEX7 depends on PEX5 binding to deliver PTS2 cargo into the peroxisome, and PEX7 also facilitates PEX5 accumulation and import of PTS1 cargo into peroxisomes [ 1 , 2 ].

This entry represents PEX7 from animals, fungi and plants. In plants it plays important roles in embryonic development, seedling establishment, and vegetative growth [ 3 ]. Budding yeast PEX7 homologue, Pas7, is necessary for import of thiolase (a PTS2-containing protein) [ 3 ]. In humans PEX7 has been linked to several diseases, such as Rhizomelic chondrodysplasia punctata 1 (RCDP1) [ 4 ] and Peroxisome biogenesis disorder 9B (PBD9B) [ 5 ].

1. The Arabidopsis peroxisomal targeting signal type 2 receptor PEX7 is necessary for peroxisome function and dependent on PEX5. Mol Biol Cell 16, 573-83
2. Interdependence of the peroxisome-targeting receptors in Arabidopsis thaliana: PEX7 facilitates PEX5 accumulation and import of PTS1 cargo into peroxisomes. Mol Biol Cell 21, 1263-71
3. PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomes. EMBO J 13, 4908-18
4. Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata. Nat. Genet. 15, 369-76
5. Identification of PEX7 as the second gene involved in Refsum disease. Am. J. Hum. Genet. 72, 471-7