Defective factor VIII causes hemophilia A

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Activation of the Factor VIIIa-Factor IXa Enzyme Complex of Blood Coagulation by Membranes Containing Phosphatidyl-L-serine. Journal of Biological Chemistry. 1996 May;271(19):11120–5. doi: 10.1074/jbc.271.19.11120.; Antonarakis SE, Kazazian HH, Tuddenham EG. Molecular etiology of factor VIII deficiency in hemophilia A. Hum Mutat. 1995;5(1):1–22. doi: 10.1002/humu.1380050102. PMID: 7728145.; Lollar P, Parker ET. Structural basis for the decreased procoagulant activity of human factor VIII compared to the porcine homolog. Journal of Biological Chemistry. 1991 Jul;266(19):12481–6. doi: 10.1016/s0021-9258(18)98924-6.; Lollar P, Parker CG. pH-dependent denaturation of thrombin-activated porcine factor VIII. Journal of Biological Chemistry. 1990 Jan;265(3):1688–92. doi: 10.1016/s0021-9258(19)40071-9.; Wion KL, Kelly D, Summerfield JA, Tuddenham EG, Lawn RM. Distribution of factor VIII mRNA and antigen in human liver and other tissues. Nature. 1985 Oct;317(6039):726–9. doi: 10.1038/317726a0. PMID: 3932885.; Salen P, Babiker HM: Hemophilia A .

Metabolites

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8



Enzyme

EC Number name full name note
3.4.21.22 coagulation factor IXa
3.4.21.5 thrombin
3.4.21.6 coagulation factor Xa


Proteins

Protein ID name full name