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
  • multiple coagulation factor deficiency 3
  • PXE-like disorder with multiple coagulation factor deficiency

More general terms

• ligase or synthetase
• transmembrane 5c protein

Database

• UniProt: http://www.uniprot.org/uniprot/P38435.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2677
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2677
• OMIM: https://mirror.omim.org/entry/137167
• OMIM: https://mirror.omim.org/entry/277450
• OMIM: https://mirror.omim.org/entry/610842