Enables peroxisome matrix targeting signal-2 binding activity. Involved in protein import into peroxisome matrix, docking. Located in cytosol and peroxisome. Human ortholog(s) of this gene implicated in Peroxisome biogenesis disorder 9B and rhizomelic chondrodysplasia punctata type 1. Orthologous to human PEX7 (peroxisomal biogenesis factor 7).
SGD Description
Peroxisomal signal receptor for peroxisomal matrix proteins; recognizes the N-terminal nonapeptide signal (PTS2); WD repeat protein; defects in human homolog cause lethal rhizomelic chondrodysplasia punctata (RCDP)