Gene Symbol: Ggcx
Description: gamma-glutamyl carboxylase
Alias: vitamin K-dependent gamma-carboxylase, peptidyl-glutamate 4-carboxylase, vitamin K gamma glutamyl carboxylase
Species: mouse
Products:     Ggcx

Top Publications

  1. Zhu A, Sun H, Raymond R, Furie B, Furie B, Bronstein M, et al. Fatal hemorrhage in mice lacking gamma-glutamyl carboxylase. Blood. 2007;109:5270-5 pubmed
    ..Heterozygous mice carrying a null mutation at the gamma-carboxylase (Ggcx) gene exhibit normal development and survival with no evidence of hemorrhage and normal functional activity of the ..
  2. Azuma K, Tsukui T, Ikeda K, Shiba S, Nakagawa K, Okano T, et al. Liver-specific ?-glutamyl carboxylase-deficient mice display bleeding diathesis and short life span. PLoS ONE. 2014;9:e88643 pubmed publisher
    ..One of its functions is as a co-factor for ?-glutamyl carboxylase (Ggcx). Conventional knockout of Ggcx causes death shortly after birth in homozygous mice...
  3. Begley G, Furie B, Czerwiec E, Taylor K, Furie G, Bronstein L, et al. A conserved motif within the vitamin K-dependent carboxylase gene is widely distributed across animal phyla. J Biol Chem. 2000;275:36245-9 pubmed
    ..The vitamin K-dependent biosynthesis of gamma-carboxyglutamic acid appears to be a highly conserved function in the animal kingdom. ..
  4. Pudota B, Hommema E, Hallgren K, McNally B, Lee S, Berkner K. Identification of sequences within the gamma-carboxylase that represent a novel contact site with vitamin K-dependent proteins and that are required for activity. J Biol Chem. 2001;276:46878-86 pubmed
  5. Czerwiec E, Begley G, Bronstein M, Stenflo J, Taylor K, Furie B, et al. Expression and characterization of recombinant vitamin K-dependent gamma-glutamyl carboxylase from an invertebrate, Conus textile. Eur J Biochem. 2002;269:6162-72 pubmed
  6. Higgins Gruber S, Mutucumarana V, Lin P, Jorgenson J, Stafford D, Straight D. Effect of vitamin K-dependent protein precursor propeptide, vitamin K hydroquinone, and glutamate substrate binding on the structure and function of {gamma}-glutamyl carboxylase. J Biol Chem. 2010;285:31502-8 pubmed publisher
    ..The two together had a greater effect than either alone. We conclude that the effect of propeptide and substrates on carboxylase controls the order of substrate binding in such a way as to ensure efficient, specific carboxylation. ..
  7. Shiba S, Ikeda K, Azuma K, Hasegawa T, Amizuka N, Horie Inoue K, et al. γ-Glutamyl carboxylase in osteoblasts regulates glucose metabolism in mice. Biochem Biophys Res Commun. 2014;453:350-5 pubmed publisher
    Vitamin K-dependent γ-glutamyl carboxylase (GGCX) is an enzyme that catalyzes the conversion of glutamic acid to gamma-carboxyglutamic acid in substrate proteins...
  8. Azuma K, Shiba S, Hasegawa T, Ikeda K, Urano T, Horie Inoue K, et al. Osteoblast-Specific γ-Glutamyl Carboxylase-Deficient Mice Display Enhanced Bone Formation With Aberrant Mineralization. J Bone Miner Res. 2015;30:1245-54 pubmed publisher
    ..In addition, it has bone-protective effects. Vitamin K functions as a cofactor of γ-glutamyl carboxylase (GGCX), which activates its substrates by carboxylation...
  9. Ferron M, LaCombe J, Germain A, Oury F, Karsenty G. GGCX and VKORC1 inhibit osteocalcin endocrine functions. J Cell Biol. 2015;208:761-76 pubmed publisher
    ..being secreted by osteoblasts in the bone extracellular matrix, OCN is γ-carboxylated by the γ-carboxylase (GGCX) on three glutamic acid residues, a cellular process requiring reduction of vitamin K (VK) by a second enzyme, a ..

More Information


  1. Li Q, Uitto J. The mineralization phenotype in Abcc6 ( -/- ) mice is affected by Ggcx gene deficiency and genetic background--a model for pseudoxanthoma elasticum. J Mol Med (Berl). 2010;88:173-81 pubmed publisher
    ..In this study, we examined the hypothesis that the GGCX gene encoding gamma-glutamyl carboxylase may interfere with the mineralization process in Abcc6 ( -/- ) mice...