Carbohydrate of the factor VIII/von Willebrand factor in von Willebrand's disease.
We have examined the plasma Factor VIII/von Willebrand factor (FVIII/vWF) molecule from 16 patients with von Willebrand's disease, and have found no evidence of a significant decrease of carbohydrate content in 15 of these patients. FVIII/vWF was isolated by preparative counter immunoelectrophoresis directly from plasma using antibody to Factor VIII-related antigen, reduced in sodium dodecyl sulfate in the presence of urea, and electrophoresed in 5% polyacrylamide gels to separate the FVIII/vWF subunit from other proteins. Duplicate gels were stained by either the periodic acid-Schiff (PAS) reaction or by Coomassie Brilliant Blue G250. The ratio of Coomassie: PAS was determined by spectrophotometric scanning of the gels. Transferrin was used as an internal reference standard. The ratio for 23 normal individuals was 2.4+/-0.38 and the observed range was 1.8-3.8. 15 patients with von Willebrand's disease fell within this range. One patient independently reported as having decreased FVIII/vWF carbohydrate was also studied by this technique. A ratio of 6.8 was found, indicative of decreased, though not absent, carbohydrate. Cold insoluble globulin did not represent a significant contaminant in these analyses. 11 of the von Willebrand's disease patients with normal FVIII/vWF carbohydrate had abnormal crossed immunoelectrophoretic patterns characterized by absence of the less anodic forms of Factor VIII-related antigen. Four patients had normal patterns. These studies indicate that an absence or decrease of PAS reactive FVIII/vWF carbohydrate is not a consistent abnormality in von Willebrand's disease.
D001779:Blood Coagulation Factors / Q000032:analysis*; D001786:Blood Glucose / Q000032:analysis*; D002241:Carbohydrates / Q000032:analysis*; D003378:Counterimmunoelectrophoresis / Q000379:methods; D004591:Electrophoresis, Polyacrylamide Gel / Q000379:methods; D005169:Factor VIII / Q000032:analysis* / Q000302:isolation & purification; D006801:Humans; D010503:Periodic Acid-Schiff Reaction / Q000379:methods; D014842:von Willebrand Diseases / Q000097:blood*; D014841:von Willebrand Factor / Q000032:analysis*
D001779:Blood Coagulation Factors; D001786:Blood Glucose; D002241:Carbohydrates; D014841:von Willebrand Factor; D005169:Factor VIII