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Abstract
Recent studies have provided substantial evidence that F.VIII/vWF exists not only
as a polymer of ~ 1 × 106 MW composed of 230,000 MW subunits but is further organized into a multimeric series
(1 to 20 × 106 MW). With an immunologic technique to isolate F.VIII/vWF directly from small volumes
of plasma of patients with vWD, the relative distribution and concentration of individual
multimeric sets was examined by agarose gel electrophoresis in the presence of SDS.
In classical vWD (type I) with decreased VIIIR:RCo, the relative distribution of multimeric
forms of FVIII/vWF was comparable to normal plasma but reduced in quantity. In “variants”
of vWD (type II) with undetectable VIIIR:RCo, the larger multimer subsets were absent,
and the smaller one present in relatively greater concentration. Large multimers as
well as VIIIR:RCo were also lacking in F.VIII/vWF prepared from normal plasma cryosupernatant.
These observations suggest the existence of different molecular abnormalities of F.VIII/vWF
underlying the two major types of vWD: reduced synthesis in type I and polymerization
defect with resultant lack of VIIIR:RCo in type II.
Abbreviations:
molecular weight (MW), factor VIII/von Willebrand factor (F.VIII/vWF), factor VIII procoagulant activity (VIII:C), ristocetin cofactor activity (VIIIR:RCo), factor VIII-related antigen (VIIIR:Ag), von Willebrand's disease (vWD), immunoglobulin G (IgG), sodium dodecyl sulfate (SDS), phenylmethylsulfonylfluoride (PMSF), immunoradiometric assay (IRMA), phosphate saline buffer (PBS), immunoglobulin M (IgM), counterimmunoelectrophoresis (CIE), void volume (Vo)To read this article in full you will need to make a payment
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Article info
Publication history
Accepted:
December 19,
1979
Received:
August 13,
1979
Footnotes
☆This work was supported by grants from INSERM (79-5-2945), DGRST (78-7-2603), and NIH (HL 16411, HL 15491, and HL 20517).
Identification
Copyright
© 1980 Published by Elsevier Inc.
