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The complex multimeric composition of factor VIII/von Willebrand factor
ZM Ruggeri and TS Zimmerman
We have analyzed the multimeric structure of factor VIII/von Willebrand
factor in plasma by sodium dodecyl sulfate electrophoresis using gels of
varying porosity and a discontinuous buffer system. Factor VIII/von
Willebrand factor bands were identified by reaction with 125I-labeled
affinity-purified antibody and subsequent autoradiography. In 1% agarose
gels, normal plasma displayed a series of sharply defined oligomers.
However, increasing the agarose concentration to 2.0% or utilizing mixtures
of 0.8% agarose--1.75% acrylamide revealed two bands of lesser intensity
interposed between the major bands. When the acrylamide concentration in
the gels was increased to 2.5%, bands with a faster mobility than IgM and
fibronectin were now evident. Type IIA von Willebrand's disease showed not
only an absence of the larger multimers but also a relative increase in
several of the newly identified bands as compared to type IIB, type I, and
normal. These studies suggest that factor VII/von Willebrand factor in IIA
von Willebrand's disease is structurally different from that in other forms
of the disorder. They also indicate that the multimeric composition of
factor VII/von Willebrand factor is more complex than can be explained by
simple linear polymerization of a single protomer.
Volume 57,
Issue 6,
pp. 1140-1143,
06/01/1981
Copyright © 1981 by The American Society of Hematology
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