PEX7 is a cytosolic receptor essential for peroxisomal import of proteins bearing C-terminal PTS2 (peroxisomal targeting signal type-2) sequences 1. The protein binds PTS2-containing cargo in the cytosol and forms a ternary complex with PEX5, which is translocated through the peroxisomal membrane via the PEX13-PEX14 docking complex 2. Recently, PEX39 was identified as a co-factor that stabilizes PEX7-cargo interactions and facilitates handover to membrane translocons 3. After cargo release in the peroxisomal matrix, PEX7 is recycled back to the cytosol through a separate retrotranslocon pathway 2. PEX7 mutations cause rhizomelic chondrodysplasia punctata (RCDP/peroxisome biogenesis disorder complementation group 11), an autosomal recessive condition characterized by developmental disabilities 1. The L292ter mutation, accounting for 50% of mutant PEX7 alleles, shows evidence of a founder effect in Caucasian populations 4. RCDP results from impaired import of PTS2-containing proteins, disrupting peroxisomal ether lipid biosynthesis and other metabolic functions confined to peroxisomes 5. Unlike broader peroxisome biogenesis disorders (Zellweger syndrome spectrum), RCDP is uniquely associated with PEX7 gene defects 5.