Reactome | factor VIII:von Willibrand factor multimer -> factor VIIIa + factor VIIIa B A3 acidic polypeptide + von Willibrand factor multimer

factor VIII:von Willibrand factor multimer -> factor VIIIa + factor VIIIa B A3 acidic polypeptide + von Willibrand factor multimer

Stable Identifier

R-HSA-158137

Type

Reaction [transition]

Species

Homo sapiens

Compartment

extracellular region

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factor VIII:von Willibrand factor multimer -> factor VIIIa + factor VIIIa B A3 acidic polypeptide + von Willibrand factor multimer

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Factor VIII complexed to von Willibrand factor in the blood is cleaved into several smaller polypeptides that remain associated. The acidic polypeptide on the aminoterminal side of the A3 domain of the light chain is released, however, and as this polypeptide mediates the association of factor VIII with von Willibrand factor, the activated factor VIII is released. While several proteases are capable of catalyzing these cleavages in vitro, only thrombin is active on factor VIII:von Willibrand factor complexes under physiological conditions (Eaton et al. 1986; Hill-Eubanks et al. 1989; Lollar et al. 1988; Pieters et al. 1989).

Literature References

PubMed ID	Title	Journal	Year
2505252	Differential proteolytic activation of factor VIII-von Willebrand factor complex by thrombin Hill-Eubanks, DC, Parker, CG, Lollar, P	Proc Natl Acad Sci U S A	1989
2502206	In situ-generated thrombin is the only enzyme that effectively activates factor VIII and factor V in thromboplastin-activated plasma Pieters, J, Lindhout, T, Hemker, HC	Blood	1989
3082357	Proteolytic processing of human factor VIII. Correlation of specific cleavages by thrombin, factor Xa, and activated protein C with activation and inactivation of factor VIII coagulant activity. Eaton, D, Rodriguez, H, Vehar, GA	Biochemistry	1986
3134349	Association of the factor VIII light chain with von Willebrand factor Lollar, P, Hill-Eubanks, DC, Parker, CG	J Biol Chem	1988