Goat GGCX Polyclonal Antibody | anti-GGCX antibody

Goat Polyclonal to Human GGCX

Cat. Num. AAA248970

• Gene Names: GGCX; VKCFD1
• Reactivity: Gibbon, Gorilla, Human, Monkey, Orangutan
• Applications: Immunohistochemistry, Western Blot, ELISA
• Purity: Immunoaffinity Purified
• Synonyms: GGCX; Polyclonal Antibody; Goat Polyclonal to Human GGCX; Anti-GGCX Antibody (C-Terminus) IHC-plus; Gamma-glutamyl carboxylase; VKCFD1; Human GGCX; anti-GGCX antibody

• Host: Goat
• Reactivity: Gibbon, Gorilla, Human, Monkey, Orangutan
• Clonality: Polyclonal
• Specificity: Human GGCX. This antibody is expected to recognize both isoforms (NP_000812.2; NP_001135741.1).
• Purity/Purification: Immunoaffinity Purified
• Form/Format: Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide
• Sequence Length: 758

• Applicable Applications for anti-GGCX antibody: Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)
• Application Notes: ELISA, IHC-P (5 ug/ml), WB (0.3 - 1 ug/ml); Usage: Western Blot: Approx 85kD band observed in Human Muscle lysates (calculated MW of 85kD according to NP_000812.2). Recommended concentration: 0.3-1 ug/ml.
• Target Species: Human
• Immunogen Description: Synthetic peptide C-PPESNPDPVHSE from the C-terminus of human GGCX (NP_000812.2; NP_001135741.1). Percent identity by BLAST analysis: Human, Gorilla, Orangutan, Gibbon, Monkey, Marmoset (100%); Elephant, Panda (83%).
• Immunogen Type: Synthetic peptide
• Immunogen: GGCX antibody was raised against synthetic peptide C-PPESNPDPVHSE from the C-terminus of human GGCX (NP_000812.2; NP_001135741.1). Percent identity by BLAST analysis: Human, Gorilla, Orangutan, Gibbon, Monkey, Marmoset (100%); Elephant, Panda (83%).
• Antigen Modification: C-Terminus
• Preparation and Storage: Store at -20 degree C. Minimize freezing and thawing.

Immunohistochemistry (IHC)

(Anti-GGCX antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.)

Western Blot (WB)

(GGCX antibody (0.3 ug/ml) staining of Human Muscle lysate (35 ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

NCBI and Uniprot Product Information

• NCBI GI #: 21361163
• NCBI GeneID: 2677
• NCBI Accession #: NP_000812.2
• NCBI GenBank Nucleotide #: NM_000821.5
• UniProt Accession #: P38435; Q14415; Q6GU45; B4DMC5; E9PEE1
• Molecular Weight: 80,989 Da
• NCBI Official Full Name: vitamin K-dependent gamma-carboxylase isoform 1
• NCBI Official Synonym Full Names: gamma-glutamyl carboxylase
• NCBI Official Symbol: GGCX
• NCBI Official Synonym Symbols: VKCFD1
• NCBI Protein Information: vitamin K-dependent gamma-carboxylase; peptidyl-glutamate 4-carboxylase
• UniProt Protein Name: Vitamin K-dependent gamma-carboxylase
• Protein Family: Vitamin K-dependent gamma-carboxylase
• UniProt Gene Name: GGCX
• UniProt Synonym Gene Names: GC
• UniProt Entry Name: VKGC_HUMAN

NCBI Description

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]

Uniprot Description

GGCX: 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. Defects in GGCX are a cause of combined deficiency of vitamin K-dependent clotting factors type 1 (VKCFD1); 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. Defects in GGCX are the cause of pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency (PXEL-MCFD). 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. Belongs to the vitamin K-dependent gamma-carboxylase family.

Protein type: EC 4.1.1.90; Ligase; Membrane protein, multi-pass; Membrane protein, integral; Lyase

Chromosomal Location of Human Ortholog: 2p12

Cellular Component: endoplasmic reticulum membrane; membrane; integral to membrane

Molecular Function: gamma-glutamyl carboxylase activity

Biological Process: cellular protein metabolic process; protein modification process; post-translational protein modification; blood coagulation; peptidyl-glutamic acid carboxylation

Disease: Pseudoxanthoma Elasticum-like Disorder With Multiple Coagulation Factor Deficiency; Vitamin K-dependent Clotting Factors, Combined Deficiency Of, 1