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vitamin K-dependent gamma-carboxylase; gamma-glutamyl carboxylase; vitamin K gamma glutamyl carboxylase (GGCX, GC)
Function:
- mediates the vitamin K-dependent carboxylation of Glu to Ca+2 binding gamma-carboxyglutamate (Gla) residues with the concomitant conversion of the reduced hydroquinone form of vitamin K to vitamin K epoxide
- vitamin K-dependent protein substrates of GGCX usually have a propeptide that binds to a high-affinity site on GGCX
- CO2, O2 & reduced vitamin K are cosubstrates
- may interact with CALU
Structure:
- monomer.
- belongs to the vitamin K-dependent gamma-carboxylase family
Compartment: endoplasmic reticulum membrane
Pathology:
- defects in GGCX are a cause of
a) multiple coagulation factor deficiency 3
b) PXE-like disorder with multiple coagulation factor deficiency
General
ligase or synthetase
transmembrane 5c protein
Properties
SIZE: entity length = 758 aa
MW = 88 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: cytoplasmic domain {1-60}
transmembrane domain {61-81}
exoplasmic loop {82-113}
MOTIF: cysteine residue {C99}
MODIFICATION: cysteine residue {C450}
transmembrane domain {114-134}
cytoplasmic loop {135-136}
transmembrane domain {137-157}
exoplasmic loop {158-292}
transmembrane domain {293-313}
cytoplasmic loop {314-361}
transmembrane domain {362-382}
exoplasmic domain {383-758}
MOTIF: cysteine residue {C450}
MODIFICATION: cysteine residue {C99}
Database Correlations
OMIM correlations
MORBIDMAP 137167
UniProt P38435
Pfam PF05090
Entrez Gene 2677
Kegg hsa:2677
References
UniProt :accession P38435