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Literature summary for 4.1.1.90 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
Search for more literature for 4.1.1.90

Protein Variants

Protein Variants Comment Organism
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

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

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
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

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
blood plasma coagulation assays Mus musculus
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CJ7 cell gamma-carboxylase gene targeting Mus musculus
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liver preparation of solubilized gamma-carboxylase Mus musculus
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R1 cell gamma-carboxylase gene targeting Mus musculus
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
FLEEL + CO2 + O2 + vitamin K1 hydroquinone a synthetic peptide substrate, assay for vitamin K–dependent carboxylase activity Mus musculus ? + vitamin K1 epoxide + H2O
-
 ?
L-glutamate + CO2 + O2 + vitamin K hydroquinone
-
 Mus musculus gamma-carboxy L-glutamate + vitamin K epoxide + H2O
-
 ?
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

Synonyms Comment Organism
gamma-carboxylase
-
 Mus musculus
gamma-glutamyl carboxylase
-
 Mus musculus

Cofactor

Cofactor Comment Organism Structure
vitamin K1
-
 Mus musculus