title: Platelet-type von Willebrand disease platelet aggregating factor: a novel functional assay of von Willebrand factor.
abstract: Blood platelets from patients with platelet-type von Willebrand disease (vWD) aggregate upon the addition of human von Willebrand factor (vWf) in the absence of ristocetin or other stimulating factors. We measured quantitatively the ability of vWf to induce directly aggregation of platelet-type vWD platelets (platelet-type vWD platelet aggregating factor [PT-PAF]). Cryoprecipitate and factor VIII concentrates were used as a source of vWf of various multimeric composition. The PT-PAF activity was dependent on the multimer size of vWf, like in the case of ristocetin cofactor (RCof) activity. However, PT-PAF activity was not equivalent to RCof activity and the relative PT-PAF/RCof ratio ranged from 1.00 to 0.18 in the materials studied. The preparations containing the higher-molecular-weight multimers had higher PT-PAF/RCof ratio. These findings suggest that PT-PAF activity is a functional expression of more highly polymerized multimers of vWf as compared with RCof activity. Measurement of PT-PAF would serve as a novel functional assay of vWf.
mesh_terms: D005169:Factor VIII / Q000032:analysis; D006801:Humans; D010974:Platelet Aggregation; D014842:von Willebrand Diseases / Q000097:blood*; D014841:von Willebrand Factor / Q000032:analysis / Q000502:physiology*
chemical_list: D014841:von Willebrand Factor; D005169:Factor VIII