Anti-GGCX Antibody (CAB1806)

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SKU:
CAB1806
Product type:
Antibody
Reactivity:
Human
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
Frequently bought together:

Description

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Antibody Name:Anti-GGCX Antibody
Antibody SKU:CAB1806
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 479-758 of human GGCX (NP_000812.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:LO2, MCF7, HeLa, Mouse liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 479-758 of human GGCX (NP_000812.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:QRIF DPRV DIVQ AAWS PFQR TSWV QPLL MDLS PWRA KLQE IKSS LDNH TEVV FIAD FPGL HLEN FVSE DLGN TSIQ LLQG EVTV ELVA EQKN QTLR EGEK MQLP AGEY HKVY TTSP SPSC YMYV YVNT TELA LEQD LAYL QELK EKVE NGSE TGPL PPEL QPLL EGEV KGGP EPTP LVQT FLRR QQRL QEIE RRRN TPFH ERFF RFLL RKLY VFRR SFLM TCIS LRNL ILGR PSLE QLAQ EVTY ANLR PFEA VGEL NPSN TDSS HSNP PESN PDPV HSEF
Gene ID:2677
Uniprot:P38435
Cellular Location:Endoplasmic reticulum membrane, Multi-pass membrane protein
Calculated MW:80kDa/87kDa
Observed MW:110kDa
Synonyms:GGCX, VKCFD1
Background:This gene encodes an integral membrane protein of the rough endoplasmic reticulum that carboxylates glutamate residues of vitamin K-dependent proteins to gamma carboxyl glutamate, a modification that is required for their activity. The vitamin K-dependent protein substrates have a propeptide that binds the enzyme, with carbon dioxide, dioxide, and reduced vitamin K acting as co-substrates. Vitamin K-dependent proteins affect a number of physiologic processes including blood coagulation, prevention of vascular calcification, and inflammation. Allelic variants of this gene have been associated with pseudoxanthoma elasticum-like disorder with associated multiple coagulation factor deficiency. Alternative splicing results in multiple transcript variants.
UniProt Protein Function: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.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 2p12

Cellular Component: endoplasmic reticulum membrane; membrane

Molecular Function:gamma-glutamyl carboxylase activity

Biological Process: blood coagulation; peptidyl-glutamic acid carboxylation; protein modification process

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

NCBI Summary:This gene encodes an integral membrane protein of the rough endoplasmic reticulum that carboxylates glutamate residues of vitamin K-dependent proteins to gamma carboxyl glutamate, a modification that is required for their activity. The vitamin K-dependent protein substrates have a propeptide that binds the enzyme, with carbon dioxide, dioxide, and reduced vitamin K acting as co-substrates. Vitamin K-dependent proteins affect a number of physiologic processes including blood coagulation, prevention of vascular calcification, and inflammation. Allelic variants of this gene have been associated with pseudoxanthoma elasticum-like disorder with associated multiple coagulation factor deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2015]
UniProt Code:P38435
NCBI GenInfo Identifier:84028279
NCBI Gene ID:2677
NCBI Accession:P38435.2
UniProt Secondary Accession:P38435,Q14415, Q6GU45, B4DMC5, E9PEE1,
UniProt Related Accession:P38435
Molecular Weight:80,989 Da
NCBI Full Name:Vitamin K-dependent gamma-carboxylase
NCBI Synonym Full Names:gamma-glutamyl carboxylase
NCBI Official Symbol:GGCX  
NCBI Official Synonym Symbols:VKCFD1  
NCBI Protein Information:vitamin K-dependent gamma-carboxylase
UniProt Protein Name:Vitamin K-dependent gamma-carboxylase
UniProt Synonym Protein Names:Gamma-glutamyl carboxylase; Peptidyl-glutamate 4-carboxylase; Vitamin K gamma glutamyl carboxylase
Protein Family:Vitamin K-dependent gamma-carboxylase
UniProt Gene Name:GGCX  
UniProt Entry Name:VKGC_HUMAN
Anti-GGCX Antibody (CAB1806)Western blot analysis of extracts of various cell lines, using GGCX antibody (CAB1806) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.

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