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Phenotypes Associated with This Genotype
Genotype
MGI:3606957
Allelic
Composition
Adamts13tm1Dgi/Adamts13tm1Dgi
Genetic
Background
involves: 129X1/SvJ * C57BL/6J * CASA/Rk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adamts13tm1Dgi mutation (1 available); any Adamts13 mutation (33 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice
• exhibit a significant decrease in survival than wildtype

hematopoietic system
N
• Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice
• 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
• 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
• 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 ID Ref(s)
Thrombotic Thrombocytopenic Purpura, Congenital; TTP 274150 J:101752


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
08/17/2016
MGI 6.05
The Jackson Laboratory