cation-dependent mannose-6-phosphate receptor; CD man-6-P receptor; CD-MPR; 46 kD mannose 6-phosphate receptor; MPR 46 (M6PR, MPR46, MPRD)

See IGF 2 receptor for cation-independent mannose-6-phosphate receptor. Function: 1) transport of phosphorylated lysosomal enzymes from Golgi & cell surface to lysosomes 2) lysosomal enzymes bearing phosphomannosyl residues bind specifically to mannose-6-phosphate receptors in the Golgi, resulting receptor-ligand complex is transported to an acidic prelyosomal compartment where the low pH mediates dissociation of the complex 3) 5-20% of newly synthesized lysosomal enzymes escape binding to the mannose-6-phosphate receptors in the Golgi & are secreted 4) binds GGA1, GGA2 GGA3 5) optimal binding in the presence of divalent cations Structure: - homodimer - extracellular domain is homologous to the repeating units (~147 AA) of the cation-independent mannose 6-phosphate receptor Compartment: lysosomal membrane Pharmacology: - target of avalglucosidase alfa-ngpt (Nexviazyme) for use in Pompe disease

General

carrier protein (transporter) glycoprotein membrane protein phosphoprotein receptor

Properties

SIZE: entity length = 277 aa MW = 31 kD COMPARTMENT: lysosome MOTIF: signal sequence {1-26} N-glycosylation site {N57} N-glycosylation site {N83} N-glycosylation site {N94} N-glycosylation site {N107} N-glycosylation site {N113} transmembrane domain {186-210} ligand-binding site FOR-BINDING-OF: mannose-6-phosphate Ser phosphorylation site {S2421} Ser phosphorylation site {S2492}

References

UniProt :accession P20645

Databases & Figures

OMIM 154540 UniProt P20645 Pfam PF02157 Kegg hsa:4074 Contents of Cytotoxic T-cell Granules