Gene and Protein Information
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	5	758	2p11.2	GGCX	gamma-glutamyl carboxylase
Mouse	5	757	6 C1	Ggcx	gamma-glutamyl carboxylase
Rat	5	758	4q31	Ggcx	gamma-glutamyl carboxylase

Previous and Unofficial Names

vitamin K-dependent gamma-carboxylase | peptidyl-glutamate 4-carboxylase | vitamin K gamma glutamyl carboxylase | VKCFD1

Database Links
Alphafold	P38435 (Hs), Q9QYC7 (Mm), O88496 (Rn)
BRENDA	4.1.1.90
CATH/Gene3D	2.60.120.10
ChEMBL Target	CHEMBL2012 (Hs), CHEMBL2744 (Rn)
DrugBank Target	P38435 (Hs)
Ensembl Gene	ENSG00000115486 (Hs), ENSMUSG00000053460 (Mm), ENSRNOG00000012975 (Rn)
Entrez Gene	2677 (Hs), 56316 (Mm), 81716 (Rn)
Human Protein Atlas	ENSG00000115486 (Hs)
KEGG Enzyme	4.1.1.90
KEGG Gene	hsa:2677 (Hs), mmu:56316 (Mm), rno:81716 (Rn)
OMIM	137167 (Hs)
Orphanet	ORPHA122083 (Hs)
Pharos	P38435 (Hs)
UniProtKB	P38435 (Hs), Q9QYC7 (Mm), O88496 (Rn)
Wikipedia	GGCX (Hs)

Enzyme Reaction
EC Number: 4.1.1.90

Substrates and Reaction Kinetics
Substrate Sp. Property Value Units Standard property Standard value Assay description Assay conditions Comments Reference glutamyl peptides Hs - -

Cofactors
Cofactor Species Comments Reference NADPH Human vitamin K hydroquinone Human

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference anisindione Hs Inhibition - - ⤷

Clinically-Relevant Mutations and Pathophysiology
Disease:  Pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency Synonyms: Body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency [Orphanet: ORPHA91135] OMIM: 610842 Orphanet: ORPHA91135
Disease:  Vitamin K-dependent clotting factors, combined deficiency of, 1; VKCFD1 Synonyms: Hereditary combined deficiency of vitamin K-dependent clotting factors [Orphanet: ORPHA98434] OMIM: 277450 Orphanet: ORPHA98434