factor VIII + von Willebrand factor multimer -> factor VIII:von Willibrand factor multimer

Stable Identifier
R-HSA-158118
Type
Reaction [binding]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Factor VIII binds to von Willebrand factor to form a complex. This complex stabilizes factor VIII, which otherwise has a very short half-life in the blood.

Factor VIII (Vehar et al. 1984) is a heterodimer containing a heavy and a light polypeptide chain, generated by the proteolytic cleavage of a single large precursor polypeptide. Several forms of the heavy chain are found in vivo, all functionally the same but differing in the amount of the B domain removed by proteolysis. The single form annotated here is the shortest one (Eaton et al. 1986; Hill-Eubanks et al. 1989).

In vitro, von Willebrand factor (Titani et al. 1986) can form complexes with factor VIII with a 1:1 stoichiometry. The complexes that form in vivo, however, involve large multimers of von Willebrand factor and varied, but always low, proportions of factor VIII (Vlot et al. 1995). A stoichiometry of one molecule of factor VIII associated with 50 of von Willebrand factor is typical in vivo, and is used here to annotate the factor VIII:von Willebrand factor complex.

Literature References
PubMed ID Title Journal Year
3524673 Amino acid sequence of human von Willebrand factor

Titani, K, Kumar, S, Takio, K, Ericsson, LH, Wade, RD, Ashida, K, Walsh, KA, Chopek, MW, Sadler, JE, Fujikawa, K

Biochemistry 1986
7756647 The affinity and stoichiometry of binding of human factor VIII to von Willebrand factor

Vlot, AJ, Koppelman, SJ, van den Berg, MH, Bouma, BN, Sixma, JJ

Blood 1995
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
6438527 Structure of human factor VIII

Vehar, GA, Keyt, B, Eaton, D, Rodriguez, H, O'Brien, DP, Rotblat, F, Oppermann, H, Keck, R, Wood, WI, Harkins, RN, Tuddenham, EG, Lawn, RM, Capon, DJ

Nature 1984
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
Participants
Participant Of
Orthologous Events
Authored
Created