protein

Recombinant Human Vitamin K-dependent gamma-carboxylase (GGCX)

MBS958004

1 mg (E Coli Derived

1795 USD

Recombinant Protein

Recombinant Human Vitamin K-dependent gamma-carboxylase (GGCX)

Vitamin K-dependent gamma-carboxylase (GGCX)

Recombinant Vitamin K-dependent gamma-carboxylase (GGCX); Vitamin K-dependent gamma-carboxylase EC= 4.1.1.90; Gamma-glutamyl carboxylase Peptidyl-glutamate 4-carboxylase Vitamin K gamma glutamyl carboxylase

vitamin K-dependent gamma-carboxylase isoform 1; Vitamin K-dependent gamma-carboxylase; vitamin K-dependent gamma-carboxylase; peptidyl-glutamate 4-carboxylase; gamma-glutamyl carboxylase; Gamma-glutamyl carboxylase; Peptidyl-glutamate 4-carboxylase; Vitamin K gamma glutamyl carboxylase

GGCX; GC

GGCX; GGCX; VKCFD1; GC

VKGC_HUMAN

E Coli or Yeast

383-758

>90%

This item requires custom production and lead time is between 5-9 weeks. We can custom produce according to your specifications.

Store at -20 degree C. For extended storage, store at -20 or -80 degree C.

Tag Information: His tagged (Host tag may vary. Please inquire for specific tag information). Species: Homo sapiens (Human)

Storage Buffer: PBS pH 7.4, 50% glycerol

21361163

NP_000812.2

NM_000821.5

P38435

87,561 Da

Gamma-carboxylation Of Protein Precursors Pathway 106233!!Gamma-carboxylation, Transport, And Amino-terminal Cleavage Of Proteins Pathway 106232!!Metabolism Of Proteins Pathway 106230!!PTM: Gamma Carboxylation, Hypusine Formation And Arylsulfatase Activation Pathway 106231!!Post-translational Protein Modification Pathway 161012!!Ubiquinone And Other Terpenoid-quinone Biosynthesis Pathway 82939!!Ubiquinone And Other Terpenoid-quinone Biosynthesis Pathway 300

This gene encodes an enzyme which catalyzes the posttranslational modification of vitamin K-dependent protein. Many of these vitamin K-dependent proteins are involved in coagulation so the function of the encoded enzyme is essential for hemostasis. Mutations in this gene are associated with vitamin K-dependent coagulation defect and PXE-like disorder with multiple coagulation factor deficiency. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2008]

Function: Mediates the vitamin K-dependent carboxylation of glutamate residues to calcium-binding gamma-carboxyglutamate (Gla) residues with the concomitant conversion of the reduced hydroquinone form of vitamin K to vitamin K epoxide. Ref.10. Catalytic activity: [Peptidyl]-4-carboxyglutamate + 2,3-epoxyphylloquinone + H2O = [peptidyl]-glutamate + CO2 + O2 + phylloquinone. Subunit structure: Monomer. May interact with CALU . By similarity. Subcellular location: Endoplasmic reticulum membrane; Multi-pass membrane protein Ref.6. Involvement in disease: Defects in GGCX are a cause of combined deficiency of vitamin K-dependent clotting factors type 1 (VKCFD1) [. MIM:277450]; also known as multiple coagulation factor deficiency III (MCFD3). VKCFD leads to a bleeding tendency that is usually reversed by oral administration of vitamin K. Ref.13 Ref.14 Ref.15 Ref.16Defects in GGCX are the cause of pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency (PXEL-MCFD) [. MIM:610842]. This syndrome is characterized by hyperlaxity of the skin involving the entire body. Important phenotypic differences with classical PXE include much more severe skin laxity with spreading toward the trunk and limbs with thick, leathery skin folds rather than confinement to flexural areas, and no decrease in visual acuity. Moreover, detailed electron microscopic analyzes revealed that alterations of elastic fibers as well as their mineralization are slightly different from those in classic PXE. Ref.17. Miscellaneous: The vitamin K-dependent protein substrates of carboxylase have usually a propeptide that binds to a high-affinity site on the carboxylase. CO2, O2 and reduced vitamin K are cosubstrates. Sequence similarities: Belongs to the vitamin K-dependent gamma-carboxylase family. Biophysicochemical propertiespH dependence:Optimum pH is 7.

1 mg (E Coli Derived)

1795 USD