Mouse Genome Informatics
hm
    Adamts13tm1Dgi/Adamts13tm1Dgi
involves: 129X1/SvJ * C57BL/6J * CASA/Rk
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice (J:101752)
• exhibit a significant decrease in survival than wildtype

hematopoietic system
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice (J:101752)
• peripheral blood smear shows schistocytes and fragmented red blood cells
• decrease in average platelet count in peripheral blood
• platelets show significantly prolonged adhesion to endothelial cells

homeostasis/metabolism
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice (J:101752)
• platelets show significantly prolonged adhesion to endothelial cells
• 40-50% increase in mean plasma von Willebrand Factor (vWF) level
• appearance of ultra-long vWF similar to the pattern observed in human thrombotic thrombocytopenic purpura (TPP)
• exhibit widespread vWF-rich and fibrin-poor hyaline thrombi in the small vessels of multiple organs

immune system
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice (J:101752)
• shiga toxin injection causes increased mortality, thrombocytopenia, microangiopathic hemolytic anemia and widespread vWF-rich and fibrin-poor hyaline thrombi in the small vessels of multiple organs and results in a syndrome closely resembling human thrombotic thrombocytopenic purpura

Mouse Models of Human Disease
OMIM IDRef(s)
Thrombotic Thrombocytopenic Purpura, Congenital; TTP 274150 J:101752