Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders

PTS1R, the human homologue of the Pichia pastoris PAS8 gene is involved in the import of the PTS1 protein into the matrix of the peroxisome. The fusion protein PAS8-PTS1R, eliminates the deficiency in the import of the PAS1 proteins in patients suffering from peroxisomal diseases. PTS1R is a soluble tripeptide and is present mainly in the cytosol but a part of the protein is bound to the peroxisomal membrane. PTS1R binds to serine-lysine-leucine peptides and antibodies against it inhibit the transport of the PTS1 proteins.