The Use of Factor VII/VWF Complex Activity Tests and SDS-Agarose Electrophoresis in the Analysis of Von Willebrand Disease
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Authors
Moossavi, Leyla
Issue Date
1988
Type
Thesis
Language
en_US
Keywords
Alternative Title
Abstract
Maintenance of a proper blood volume in a mammal is dependent on
the formation of clots at sites where a blood vessel has been
injured. Coagulation requires a series of clotting factors present in
plasma as well as adhesion of platelets to the subendothelial wall
of the damaged vessel. Defects in either of these components can
result in bleeding disorders. The adhesion of platelets to
subendothelial tissue depends on a plasma glycoprotein called von
Willebrand Factor, which occurs as a series of multimers with
molecular weights of 1-20 x 10 6. Genetically transmitted
quantitative and qualitative deficiencies in von Willebrand Factor
are collectively referred to as von Willebrand Disease. Type I, the
most common form of vWD, is characterized by variably decreased
levels of apparently normal vWF. Type II, or variant vWD, appears to
be more of a qualitative defect and is characterized by an absence of
the larger molecular-weight forms or multimers of vWF (Ruggeri,
1987). Type III is the most rarely seen type. It is considered the
"severe"form of vWD and is a recessive autosomal trait. To diagnose
among the different forms of the disease, a technique based on
discontinuous sodium dodecyl sulfate (SDS) agarose gel
electrophoresis was developed. Different numbers of von Willebrand
Factor multimers could be detected by directly staining gels with
immunoglobulins, an avidin-biotin peroxidase complex, and the
noncarcinogenic substrate 4-chloro-1-naphthol. Thirteen patients
were screened for vWD. Of these one had Type I, two had normal
clotting activity and the remainder were diagnosed as Type II. This
study of normal blood and that of patients with vWD indicated that
discontinuous sodium dodecyl sulfate (SDS) agarose gel
electrophoresis, in combination with routine coagulation tests,
allows precise detection and diagnosis of vWD.
Description
vi, 22 p.
Citation
Publisher
Kalamazoo College
License
U.S. copyright laws protect this material. Commercial use or distribution of this material is not permitted without prior written permission of the copyright holder.