Zebrafish Von Willebrand Factor Page: 12
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patients with severe vWD type 3, a quantitative form of vWD [83]. The investigators of
this particular model are able to visualize thrombus formation in these mice; however,
the procedure is invasive and requiring a surgical procedure, in which mesentery must
be "exteriorized" [83]. In addition to performing a surgical procedure the investigators
also collected and labeled platelets, which were then injected back into the mouse to
visualize clot formation [83]. While there are several models for the type 3 vWD there is
a murine model for the quantitative vWD type 1 vWD using the RIIIS/J inbred mouse,
which has been treated successfully using DDAVP [84]. This particular model does
provide some similarities between human vWD, and while there is survival of neonates
homozygous for VWF knockout there is a reduction in survival rate. The homozygous
knockout of VWF provides a model that is absent of VWF, important for the
investigation of treatment for type 3 vWD individuals. Thus, although this mouse model
provides a setting for the examination of the defects in VWF, disadvantages such as low
fecundity and lack of easy visualization of thrombus formation and manipulation still
remain. In addition to the mouse model the canine and porcine model has also been
used for the investigation of vWD. Both of these animal models like the murine model
are used for the study of vWD type 3 [85]. The canine model has also been used for
the study of a-granule formation in endothelial cells [86]. The porcine model has been
used in bone marrow transplantation studies and the effects of VWF on atherosclerosis
[87; 88].
As for the study of vWD type 2 there are few models for this qualitative form.
One such model is the found in German shorthair pointers, while rare, they do have the
characteristic reduction of the higher molecular weight multimers similar to type 2A [89].12
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Carrillo, Maira M. Zebrafish Von Willebrand Factor, dissertation, August 2012; Denton, Texas. (https://digital.library.unt.edu/ark:/67531/metadc177228/m1/20/: accessed April 25, 2024), University of North Texas Libraries, UNT Digital Library, https://digital.library.unt.edu; .