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The RIIIS/J inbred mouse strain as a model for von Willebrand disease
JD Sweeney, EK Novak, M Reddington, KH Takeuchi and RT Swank
Department of Molecular and Cellular Biology, Roswell Park Memorial
Institute, Buffalo, NY 14263.
Mice of the RIIIS/J inbred strain have prolonged bleeding times (greater
than 15 minutes) after experimental injury when compared with normal
C57BL/6J mice (1.8 minutes) and other strains of mice. The prolonged
bleeding time was accompanied by normal platelet counts. Platelet
aggregation with collagen and agglutination with ristocetin were not
significantly altered in RIIIS/J mice. Also, platelets from RIIIS/J mice
had normal serotonin content and normal numbers of dense granules by
electron microscopy. Thus, the bleeding abnormality is not due to platelet
storage pool deficiency as has been found in several other mouse mutants.
The activated partial thromboplastin time (APTT) which plasma from RIIIS/J
mice was prolonged compared with normal mice, and factor VIII:C activity
and von Willebrand antigen levels were one half to one third that of normal
mouse plasma. Factor XI activity was also significantly deficient (levels
at 42% to 64% of normal). Plasma of RIIIS/J mice contained the full
complement of multimers of von Willebrand factor, although each multimer
was lower in concentration compared with that in normal mice. Platelet
alpha-granule von Willebrand antigen levels were similar to those of normal
mice. The prolonged bleeding time of RIIIS/J mice was corrected by
treatment with desmopressin. Heterozygous C57BL/6J x RIIIS/J F1 animals had
low plasma von Willebrand antigen levels like the RIIIS/J parent and had
variable bleeding times. Inheritance of the bleeding tendency was as an
incomplete dominant, autosomal trait. These data indicate the RIIIS/J
strain is a suitable animal model for type IA von Willebrand disease.
Volume 76,
Issue 11,
pp. 2258-2265,
12/01/1990
Copyright © 1990 by The American Society of Hematology
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