[Biology of von Willebrand factor and pathogenesis of von Willebrand disease].
Rinsho Ketsueki
; 65(8): 756-763, 2024.
Article
en Ja
| MEDLINE
| ID: mdl-39231704
ABSTRACT
Von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative and qualitative abnormalities of von Willebrand factor (VWF), a multimeric glycoprotein that is the largest of its kind in plasma and is also found in platelet alpha granules and Weibel-Palade bodies of endothelial cells. VWF plays two roles in hemostasis (1) primary hemostasis via adhesion of platelet GPIb to subendothelial connective tissue and (2) stabilization of coagulation factor VIII. The pathological classification proposed by the International Society of Thrombosis and Haemostasis (ISTH) in 1994 divided VWF into three major categories based on the results of VWFRCo, VWFAg, and multimer analysis. Recent genetic analysis and molecular and cellular analysis of abnormal VWF have revealed a molecular basis for the dominant inheritance form of VWD.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Enfermedades de von Willebrand
/
Factor de von Willebrand
Límite:
Animals
/
Humans
Idioma:
Ja
Revista:
Rinsho Ketsueki
Año:
2024
Tipo del documento:
Article