Abstract
Recent studies have provided substantial evidence that F. VIII/vWF exists not only as a polymer of approximately 1 x 10(6) MW composed of 230,000 MW subunits but is further organized into a multimeric series (1 to 20 x 10(6) 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.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.