Mouse anti-Human Factor VIII Monoclonal Antibody | anti-FVIII antibody

Factor VIII (Coagulation Factor VIII, FVIII, F8 Protein, F8B, F8C, Antihemophilic Factor, AHF, Dxs1253e, HEMA, Procoagulant Component) (MaxLight 650)

Cat. Num. AAA6222105

• Gene Names: F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• Reactivity: Human
• Applications: FLISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: Factor VIII; Monoclonal Antibody; Factor VIII (Coagulation Factor VIII; FVIII; F8 Protein; F8B; F8C; Antihemophilic Factor; AHF; Dxs1253e; HEMA; Procoagulant Component) (MaxLight 650); anti-FVIII antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2b,k
• Clone Number: 1E8
• Specificity: Recognizes human F8.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight650.

• Applicable Applications for anti-FVIII antibody: FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa213-312 from human F8 (NP_000123) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: KFILLFAVFDEGKSWHSETKNSLMQDRDAASARAWPKMHTVNGYVNRSLPGLIGCHRKSVYWHVIGMGTTPEVHSIFLEGHTFLVRNHRQASLEISPITF
• Conjugate: MaxLight650
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Related Product Information for anti-FVIII antibody

Von Willebrand factor (VWF) is a blood glycoprotein required for normal hemostasis. VWF mediates the adhesion of platelets to sites of vascular damage by binding to specific platelet membrane glycoproteins and to constituents of exposed connective tissue. It also induces expression of the fibrinogen receptor and thus initiates platelet-platelet interaction. It is a carrier protein for blood clotting factor VIII and also stabilizes factor VIII and may serve to transport it to sites of vessel injury. It is assembled from identical approximately 250kD subunits into disulfide-linked multimers that may be greater 20,000kD. Mutations in VWD disrupt the complex biosynthetic process at several steps to impair the assembly, intracellular targeting, or secretion of VWF multimers. Deficiency of VWF leads to von Willebrand disease (VWD), and a large number of other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic-uremic syndrome. It is mapped on human chromosome 12p13.3.

Product Categories/Family for anti-FVIII antibody

Antibodies; Coagulation Factors

NCBI and Uniprot Product Information

• NCBI GI #: 4503647
• NCBI GeneID: 2157
• NCBI Accession #: NP_000123
• NCBI GenBank Nucleotide #: NM_000132
• UniProt Accession #: P00451; Q14286; Q5HY69
• Molecular Weight: 24,641 Da
• NCBI Official Full Name: coagulation factor VIII isoform a preproprotein
• NCBI Official Synonym Full Names: coagulation factor VIII
• NCBI Official Symbol: F8
• NCBI Official Synonym Symbols: AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• NCBI Protein Information: coagulation factor VIII
• UniProt Protein Name: Coagulation factor VIII
• Protein Family: Factor VIII intron 22 protein
• UniProt Gene Name: F8
• UniProt Synonym Gene Names: F8C; AHF
• UniProt Entry Name: FA8_HUMAN

NCBI Description

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 Description

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.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: extracellular space; plasma membrane; extracellular region

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