Cell Biology Antibodies 14

Anti-F8 Antibody (CAB13979)

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SKU:
CAB13979
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-F8 Antibody
Antibody SKU:CAB13979
Antibody Size:20uL, 50uL, 100uL
Application:IHC
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-216 of human F8 (NP_063916.1).
Application:IHC
Recommended Dilution:IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-216 of human F8 (NP_063916.1).
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:MRIQ DPGK VFFG NVDS SGIK HNIF NPPI IARY IRLH PTHY SIRS TLRM ELMG CDLN SCSM PLGM ESKA ISDA QITA SSYF TNMF ATWS PSKA RLHL QGRS NAWR PQVN NPKE WLQV DFQK TMKV TGVT TQGV KSLL TSMY VKEF LISS SQDG HQWT LFFQ NGKV KVFQ GNQD SFTP VVNS LDPP LLTR YLRI HPQS WVHQ IALR MEVL GCEA QDLY
Gene ID:2157
Uniprot:P00451
Cellular Location:Secreted, extracellular space
Calculated MW:24kDa/267kDa
Observed MW:
Anti-F8 AntibodyImmunohistochemistry - F8 Rabbit pAb (CAB13979)
Synonyms:F8, AHF, DXS1253E, F8B, F8C, FVIII, HEMA
Background:This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder.
UniProt Protein Function:F8: Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. Defects in F8 are the cause of hemophilia A (HEMA). A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non- functional; i.e. the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein. Belongs to the multicopper oxidase family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: extracellular space; extracellular region; plasma membrane

Molecular Function:protein binding; copper ion binding; serine-type endopeptidase activity; oxidoreductase activity

Biological Process: platelet activation; platelet degranulation; acute-phase response; proteolysis; blood coagulation; blood coagulation, intrinsic pathway

Disease: Hemophilia A; Factor Viii Deficiency

NCBI Summary:This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. [provided by RefSeq, Jul 2008]
UniProt Code:P00451
NCBI GenInfo Identifier:119767
NCBI Gene ID:2157
NCBI Accession:P00451.1
UniProt Secondary Accession:P00451,Q14286, Q5HY69,
UniProt Related Accession:P00451
Molecular Weight:24,641 Da
NCBI Full Name:Coagulation factor VIII
NCBI Synonym Full Names:coagulation factor VIII, procoagulant component
NCBI Official Symbol:F8  
NCBI Official Synonym Symbols:AHF; F8B; F8C; HEMA; FVIII; DXS1253E  
NCBI Protein Information:coagulation factor VIII; factor VIII F8B; antihemophilic factor; coagulation factor VIIIc
UniProt Protein Name:Coagulation factor VIII
UniProt Synonym Protein Names:Antihemophilic factor; AHF; Procoagulant componentCleaved into the following 4 chains:Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain
Protein Family:Factor VIII intron 22 protein
UniProt Gene Name:F8  
UniProt Entry Name:FA8_HUMAN
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