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Literature summary extracted from

  • Zhu, A.; Sun, H.; Raymond, R.M. Jr.; Furie, B.C.; Furie, B.; Bronstein, M.; Kaufman, R.J.; Westrick, R.; Ginsburg, D.
    Fatal hemorrhage in mice lacking (gamma)-glutamyl carboxylase (2007), Blood, 109, 5270-5275.
    View publication on PubMedView publication on EuropePMC

Protein Variants

EC Number Protein Variants Comment Organism
4.1.1.90 additional information analysis of a Ggcx+/- intercross reveals a partial developmental block with only 50% of expected Ggcx-/- offspring surviving to term, with the latter animals dying uniformly at birth of massive intra-abdominal hemorrhage. This phenotype closely resembles the partial midembryonic loss and postnatal hemorrhage previously reported for both prothrombin and factor V (F5)–deficient mice. Ggcx-/-, dying uniformly at birth of massive intra-abdominal hemorrhage. 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 vitamin K–dependent clotting factors IX, X, and prothrombin Mus musculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
4.1.1.90 endoplasmic reticulum transmembrane protein Mus musculus 5783
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.1.1.90 additional information Mus musculus an essential posttranslational modification required for the biological activity of a number of proteins, including proteins involved in blood coagulation and its regulation ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
4.1.1.90 Mus musculus
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.1.1.90 blood plasma coagulation assays Mus musculus
-
4.1.1.90 CJ7 cell gamma-carboxylase gene targeting Mus musculus
-
4.1.1.90 liver preparation of solubilized gamma-carboxylase Mus musculus
-
4.1.1.90 R1 cell gamma-carboxylase gene targeting Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.1.1.90 FLEEL + CO2 + O2 + vitamin K1 hydroquinone a synthetic peptide substrate, assay for vitamin K–dependent carboxylase activity Mus musculus ? + vitamin K1 epoxide + H2O
-
 ?
4.1.1.90 L-glutamate + CO2 + O2 + vitamin K hydroquinone
-
 Mus musculus gamma-carboxy L-glutamate + vitamin K epoxide + H2O
-
 ?
4.1.1.90 additional information an essential posttranslational modification required for the biological activity of a number of proteins, including proteins involved in blood coagulation and its regulation Mus musculus ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
4.1.1.90 gamma-carboxylase
-
 Mus musculus
4.1.1.90 gamma-glutamyl carboxylase
-
 Mus musculus

Cofactor

EC Number Cofactor Comment Organism Structure
4.1.1.90 vitamin K1
-
 Mus musculus