[Structure and function of the factor VIII/von Willebrand factor complex].
In the blood plasma factor VIII is bound to the von Willebrand factor. The primary structure of the two proteins were clarified by gene clonation. Factor VIII descends from a precursor protein with 2,351 amino acids by splitting of 19 amino acid residues and is activated by partial proteolysis. In the blood coagulation factor VIII acts as co-factor for the activation of factor X by factor IX in the presence of phospholipids and Ca++ within the intrinsic coagulation system. The formation of the von Willebrand factor takes place by splitting of 22 and 741 amino acid residues, respectively, from pre-pro-von Willebrand factor via pro-von Willebrand factor. The subunits of the von Willebrand factor consist od 2,050 amino acid residues. In the blood plasma the von Willebrand factor is existing as a mixture of multimeres. Receptors of the von Willebrand factor on the thrombocytic membrane are the glycoproteins GPIb and GPIIb/GPIIIa, by means of which the adhesion of thrombocytes at the subendoethelium of the vascular wall and the aggregation of thrombocytes are mediated.
Müller
G
G
Klinik und Poliklinik für Innere Medizin, Martin-Luther-Universität Halle-Wittenberg.
D000596:Amino Acids / Q000378:metabolism; D003001:Cloning, Molecular; D005169:Factor VIII / Q000502:physiology*; D006801:Humans; D010455:Peptides / Q000378:metabolism; D010980:Platelet Membrane Glycoproteins; D011956:Receptors, Cell Surface / Q000502:physiology*; D014841:von Willebrand Factor / Q000502:physiology*
D000596:Amino Acids; D010455:Peptides; D010980:Platelet Membrane Glycoproteins; D011956:Receptors, Cell Surface; D014841:von Willebrand Factor; C028254:von Willebrand factor receptor; D005169:Factor VIII
Struktur und Funktion des Faktor VIII/von-Willebrand-Faktor-Komplexes.