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

Introduction

See IGF 2 receptor for cation-independent mannose-6-phosphate receptor.

Function

• transport of phosphorylated lysosomal enzymes from Golgi & cell surface to lysosomes
• 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
• 5-20% of newly synthesized lysosomal enzymes escape binding to the mannose-6-phosphate receptors in the Golgi & are secreted
• binds GGA1, GGA2 GGA3
• 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

More general terms

• receptor
• carrier protein (transporter)
• membrane protein
• phosphoprotein
• glycoprotein

Additional terms

• mannose-6-phosphate

Database

• UniProt: http://www.uniprot.org/uniprot/P20645.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4074
• OMIM: https://mirror.omim.org/entry/154540