Target General Infomation
Target ID
T97174
Former ID
TTDS00481
Target Name
Mannose-6-phosphate receptor
Gene Name
M6PR
Synonyms
46kDa mannose 6-phosphate receptor; CD-MPR; CDMan-6-P receptor; MPR 46; MPR46; MPRD; M6PR
Target Type
Successful
Disease Pompe's disease; Type 2 glycogen storage disease [ICD9:271; ICD10: E74.02, E74.0]
Function
Transport of phosphorylated lysosomal enzymes from the Golgi complex and the cell surface to lysosomes. Lysosomal enzymes bearing phosphomannosyl residues bind specifically to mannose-6- phosphate receptors in the Golgi apparatus and the resulting receptor-ligand complex is transported to an acidic prelyosomal compartment where the low pH mediates the dissociation of the complex.
UniProt ID
Sequence
MFPFYSCWRTGLLLLLLAVAVRESWQTEEKTCDLVGEKGKESEKELALVKRLKPLFNKSF
ESTVGQGSDTYIYIFRVCREAGNHTSGAGLVQINKSNGKETVVGRLNETHIFNGSNWIML
IYKGGDEYDNHCGKEQRRAVVMISCNRHTLADNFNPVSEERGKVQDCFYLFEMDSSLACS
PEISHLSVGSILLVTFASLVAVYVVGGFLYQRLVVGAKGMEQFPHLAFWQDLGNLVADGC
DFVCRSKPRNVPAAYRGVGDDQLGEESEERDDHLLPM
Drugs and Mode of Action
Drug(s) Alglucosidase alfa Drug Info Approved Pompe's disease; Type 2 glycogen storage disease [528715], [538381], [542561]
Inhibitor 1-3 Sugar Ring of Pentamannosyl 6-Phosphate Drug Info [551393]
Alpha-D-Mannose-6-Phosphate Drug Info [551393]
Binder Alglucosidase alfa Drug Info [537500]
Pathways
KEGG Pathway Lysosome
Phagosome
Reactome Lysosome Vesicle Biogenesis
References
Ref 5287152006 drug approvals: finding the niche. Nat Rev Drug Discov. 2007 Feb;6(2):99-101.
Ref 538381FDA Approved Drug Products from FDA Official Website. 2009. Application Number: (BLA) 125141.
Ref 542561(http://www.guidetopharmacology.org/) Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 7561).
Ref 537500Early Treatment with Alglucosidase Alfa Prolongs Long Term Survival of Infants with Pompe Disease. Pediatr Res. 2009 Jun 17.
Ref 551393How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6.

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