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  • Title: Measurement of von Willebrand factor-specific mRNA and release and storage of von Willebrand factor from endothelial cells of dogs with type-I von Willebrand's disease.
    Author: Meinkoth JH, Meyers KM.
    Journal: Am J Vet Res; 1995 Dec; 56(12):1577-85. PubMed ID: 8599517.
    Abstract:
    OBJECTIVE: To characterize the cellular basis of the plasma von Willebrand factor (vWf) deficiency in Doberman Pinschers with type-1 von Willebrand's disease (vWd). ANIMALS: Five Doberman Pinschers with type-I vWd and 5 clinically normal dogs used as controls. PROCEDURE: Vascular endothelial cell cultures were used to measure constitutive vWf release, thrombin-stimulated vWf release, baseline intracellular vWf concentration, and vWf mRNA expression. RESULTS: Cells cultured from vWd-affected dogs were morphologically indistinguishable from cells cultured from control dogs, but had reductions in constitutive vWf release (6.5-fold) and vWf mRNA content (fivefold) that correlated to the reduction in plasma vWf concentration (sixfold) in these dogs. The 9.0-kb, canine vWf message was identified, using a polymerase chain reaction-amplified segment of the canine vWf gene and was similar in size to the human vWf message. The vWd cells also had reductions in baseline intracellular vWf concentration (15.6-fold) and thrombin-stimulated vWf release (14.5-fold). Additionally, it was observed that normal canine endothelial cells from different anatomic locations were heterogeneous with respect to vWf expression. CONCLUSIONS: These findings suggest that the plasma vWf deficit in dogs with type-I vWd results from decreased endothelial cell production of vWf resulting from either decreased transcription of the vWf gene or abnormalities in mRNA processing/stability. This is similar to findings in human beings with type-I vWd.
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