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Mouse Anti-F8 Recombinant Antibody (CBXF-3549) (CBMAB-F2090-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXF-3549
Antibody Isotype
IgG2b, κ
Application
ELISA

Basic Information

Immunogen
Partial recombinant corresponding to aa213-312 from human F8 (NP_000123) with GST tag
Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Coagulation Factor VIII
Introduction
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.
Entrez Gene ID
2157
UniProt ID
P00451
Alternative Names
Coagulation Factor VIII; Coagulation Factor VIII, Procoagulant Component; Antihemophilic Factor; F8C; AHF; Coagulation Factor VIII A1 Domain; Coagulation Factor VIII C2 Domain; Coagulation Factor VIIIc; Procoagulant Component;
Research Area
Factor VIII, along with calcium and phospholipid, acts as a cofactor for F9/factor IXa when it converts F10/factor X to the activated form, factor Xa.
Biological Process
Acute-phase response Source: UniProtKB-KW
Blood coagulation Source: ProtInc
Blood coagulation, intrinsic pathway Source: GO_Central
Cellular Location
Extracellular space
Involvement in disease
Hemophilia A (HEMA):
The disease is caused by variants affecting the gene represented in this entry. 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. 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.
PTM
Sulfation on Tyr-1699 is essential for binding vWF.
More Infomation

Han, J. P., Song, D. W., Lee, J. H., Lee, G. S., & Yeom, S. C. (2021). Novel severe hemophilia a mouse model with factor VIII intron 22 inversion. Biology, 10(8), 704.

Collins, A. E., Curry, N., Raza‐Burton, S., Bignell, P., Davies, C., Joffe, M., ... & Desborough, M. J. (2021). Factor VIII levels and bleeding according to factor 8 (F8) mutation in pregnant carriers of haemophilia A: a multicentre retrospective cohort study. British journal of haematology, 193(2), 397-400.

Simioni, P., Cagnin, S., Sartorello, F., Sales, G., Pagani, L., Bulato, C., ... & Castoldi, E. (2021). Partial F8 gene duplication (factor VIII Padua) associated with high factor VIII levels and familial thrombophilia. Blood, 137(17), 2383-2393.

Gong, J., Chung, T. H., Zheng, J., Zheng, H., & Chang, L. J. (2021). Transduction of modified factor VIII gene improves lentiviral gene therapy efficacy for hemophilia A. Journal of Biological Chemistry, 297(6).

Mamoor, S. (2021). Differential expression of coagulation factor VIII in cancers of the breast.

Chen, C. Y., Tran, D. M., Cavedon, A., Cai, X., Rajendran, R., Lyle, M. J., ... & Miao, C. H. (2020). Treatment of hemophilia A using factor VIII messenger RNA lipid nanoparticles. Molecular Therapy-Nucleic Acids, 20, 534-544.

Chen, H., Shi, M., Gilam, A., Zheng, Q., Zhang, Y., Afrikanova, I., ... & Chen-Tsai, R. Y. (2019). Hemophilia A ameliorated in mice by CRISPR-based in vivo genome editing of human Factor VIII. Scientific reports, 9(1), 1-15.

Neumeyer, J., Lin, R. Z., Wang, K., Hong, X., Hua, T., Croteau, S. E., ... & Melero-Martin, J. M. (2019). Bioengineering hemophilia A–specific microvascular grafts for delivery of full-length factor VIII into the bloodstream. Blood advances, 3(24), 4166-4176.

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For research use only. Not intended for any clinical use.

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