title: Carbohydrate deficiency of the factor VIII/von Willebrand factor Protein in von Willebrand's disease variants.
abstract: Study of the normal human factor VIII/von Willebrand factor reveals a macromolecular glycoprotein composed of apparently identical subunits. This purified glycoprotein has procoagulant, antigen, and von Willebrand factor activities. In three patients with a variant of the von Willebrand's disease syndrome, their factor VIII/von Willebrand factor protein was present in normal amounts and had normal procoagulant and antigen activities; however, this protein was deficient in both carbohydrate and von Willebrand factor activity. The carbohydrate portion of the factor VIII/von Willebrand factor glycoprotein is of major importance in its interactions with platelets or the blood vessel wall, or both.
mesh_terms: D000941:Antigens; D001779:Blood Coagulation Factors / Q000032:analysis*; D002241:Carbohydrates / Q000032:analysis; D006023:Glycoproteins / Q000097:blood; D013329:Structure-Activity Relationship; D014842:von Willebrand Diseases / Q000097:blood*; D014841:von Willebrand Factor / Q000032:analysis* / Q000276:immunology / Q000502:physiology
chemical_list: D000941:Antigens; D001779:Blood Coagulation Factors; D002241:Carbohydrates; D006023:Glycoproteins; D014841:von Willebrand Factor