Rabbit Coagulation Factor VIII (Cleaved form, C-Term) Polyclonal Antibody | anti-F8 antibody

Rabbit anti CD117/c-Kit (CT) Polyclonal antibody

Cat. Num. AAA462033

• Gene Names: F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• Reactivity: Human, Mouse, Rat, Canine
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: The Rabbit IgG is purified by Epitope Affinity Purification.
• Synonyms: Coagulation Factor VIII (Cleaved form; C-Term); Polyclonal Antibody; Rabbit anti CD117/c-Kit (CT) Polyclonal antibody; Rabbit anti--c-Kit (CT); Kit Protein; c-Kit; CD117 antigen; SCFR; PBT; anti-F8 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat, Canine
• Clonality: Polyclonal
• Specificity: This antibody recognizes CD17/c-Kit protein. It crossreacts to human, mouse and rat. The other species were not tested.
• Purity/Purification: The Rabbit IgG is purified by Epitope Affinity Purification.
• Form/Format: This affinity purified antibody is supplied in sterile Phosphatebuffered saline (pH7.2) containing antibody stabilizer
• Concentration: Size: 100 ug/200 ul (varies by lot)

• Applicable Applications for anti-F8 antibody: ELISA, Western Blot, Immunohistochemistry
• Application Notes: Western Blot: 0.1-1 ug/ml; ELISA: 0.01-0.1 ug/ml; Immunoprecipitation: 2-5 ug/ml; Immunohistochemistry: 2-5 ug/ml
• Antigen Preparation: A synthetic peptide derived from N-terminus of Kit protein. This sequence is identical in human, rat, mouse and dog origins.
• Positive Control: Jurkat cell.
• Cellular Location: Membrane/Cytoplasmic
• Preparation and Storage: The antibodies are stable for 12 months from date of receipt when stored at -20 degree C to -70 degree C. The antibodies can be stored at 2 degree C-8 degree C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.

Related Product Information for anti-F8 antibody

The c-Kit proto-oncogene encodes a 145 kDa transmembrane receptor (CD117) with tyrosine kinase activity that is structurally related to platelet-derived growth factor. C-Kit/CD117 antigen is expressed on almost all hematopoietic stem and progenitor cells. c-Kit regulates a variety of biological responses including cell proliferation, apoptosis, chemotaxis and adhesion. Ligand binding to the extracellular domain leads to autophosphorylation on several tyrosine residues within the cytoplasmic domain and activation. Mutations in c-Kit have been found to be important for tumor growth and progression in a variety of cancers including mast cell diseases, gastrointestinal stromal tumor, acute my+Q24eloid leukemia, Ewing sarcoma and lung cancer. C-kit plays an important role in hematopoiesis, melanogenesis, and gametogenesis.

References

Li J et al. Expression of c-Kit receptor tyrosine kinase and effect on beta-cell development in the human fetal pancreas. Am J Physiol Endocrinol Metab 293:E475-83 (2007).

NCBI and Uniprot Product Information

• NCBI GI #: 182383
• NCBI GeneID: 2157
• NCBI Accession #: AAA52420.1
• NCBI GenBank Nucleotide #: M88628.1
• UniProt Accession #: P00451; Q5HY69; A0A2G2; A0A2G3; A0A2G4; A0A2G5; A0A2G6; A0A2G8; A0A2G9; A0FJ18; A0FJ19; A0FJ20
• Molecular Weight: 80 kDa, 265 kDa
• NCBI Official Full Name: coagulation factor VIII
• NCBI Official 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; OTTHUMP00000024269; OTTHUMP00000196174; antihemophilic factor; coagulation factor VIIIc
• UniProt Protein Name: Coagulation factor VIII
• Protein Family: Coagulation factor
• UniProt Gene Name: F8
• UniProt Synonym Gene Names: F8C
• 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]

Uniprot Description

Function: 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.

Subunit structure: Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation. Ref.10 Ref.11

Subcellular location: Secreted › extracellular space.

Domain: Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.

Post-translational modification: Sulfation on Tyr-1699 is essential for binding vWF.

Involvement in disease: Defects in F8 are the cause of hemophilia A (HEMA) [

MIM:306700]. 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. Note=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. Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.36 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45 Ref.46 Ref.47 Ref.48 Ref.49 Ref.50 Ref.51 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.57 Ref.58 Ref.59 Ref.60 Ref.61 Ref.62 Ref.63 Ref.64 Ref.65 Ref.66 Ref.67 Ref.68 Ref.69 Ref.70 Ref.71 Ref.72 Ref.73 Ref.74 Ref.75 Ref.76 Ref.77 Ref.78 Ref.79 Ref.80 Ref.82 Ref.83 Ref.84 Ref.85 Ref.86 Ref.87 Ref.88 Ref.89 Ref.90 Ref.91 Ref.92 Ref.93 Ref.94 Ref.95 Ref.96 Ref.97

Pharmaceutical use: Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A.

Sequence similarities: Belongs to the multicopper oxidase family.Contains 3 F5/8 type A domains.Contains 2 F5/8 type C domains.Contains 6 plastocyanin-like domains.

Mass spectrometry: Molecular mass is 1367.6 Da from positions 356 - 378. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 1407.4 Da from positions 356 - 378. Determined by ESI. Sulfated. Ref.8Molecular mass is 2975.4 Da from positions 400 - 424. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 3024 Da from positions 727 - 752. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 3104 Da from positions 727 - 752. Determined by ESI. Monosulfated. Ref.8Molecular mass is 3183.5 Da from positions 727 - 752. Determined by ESI. Disulfated. Ref.8Molecular mass is 3262.5 Da from positions 727 - 752. Determined by ESI. Trisulfated. Ref.8Molecular mass is 2460.9 Da from positions 1672 - 1692. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 2540.7 Da from positions 1672 - 1692. Determined by ESI. Sulfated. Ref.8Molecular mass is 2000.7 Da from positions 1691 - 1708. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 2080.7 Da from positions 1691 - 1708. Determined by ESI. Sulfated. Ref.8