Phenotypes associated with this allele

• Allele Symbol: Adamts13^tm1Dgi
• Allele Name: targeted mutation 1, David Ginsburg
• Allele ID: MGI:3605825
• Summary: 5 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Adamts13^tm1Dgi/Adamts13^tm1Dgi	B6.129X1-Adamts13^tm1Dgi	MGI:4834629
hm2	Adamts13^tm1Dgi/Adamts13^tm1Dgi	involves: 129X1/SvJ * C57BL/6J	MGI:3606955
hm3	Adamts13^tm1Dgi/Adamts13^tm1Dgi	involves: 129X1/SvJ * C57BL/6J * CASA/Rk	MGI:3606957
cx4	Adamts13^tm1Dgi/Adamts13^tm1Dgi Vwf^tm1Wgr/Vwf^tm1Wgr	B6.129-Adamts13^tm1Dgi Vwf^tm1Wgr	MGI:4834630
cx5	Adamts13^tm1Dgi/Adamts13^tm1Dgi Vwf^tm1Wgr/Vwf^tm1Wgr	involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * CASA/Rk	MGI:4834624

Genotype
MGI:4834629

hm1

• Allelic Composition: Adamts13^tm1Dgi/Adamts13^tm1Dgi
• Genetic Background: B6.129X1-Adamts13^tm1Dgi

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

homeostasis/metabolism

increased susceptibility to induced thrombosis ( J:133469 )

• following FeCl3 injury, mice exhibit a reduced time to clot formation and vessel occlusion compared with similarly treated wild-type mice

Genotype
MGI:3606955

hm2

• Allelic Composition: Adamts13^tm1Dgi/Adamts13^tm1Dgi
• Genetic Background: involves: 129X1/SvJ * C57BL/6J

normal phenotype

no abnormal phenotype detected ( J:101752 )

• Background Sensitivity: exhibit normal development, fertility, survival, blood smears and a similar response to shiga toxin as wildtype, even though von Willebrand Factor (vWF)-cleaving activity is lost, unlike on a background that contains CASA/Rk

Genotype
MGI:3606957

hm3

• Allelic Composition: Adamts13^tm1Dgi/Adamts13^tm1Dgi
• Genetic Background: involves: 129X1/SvJ * C57BL/6J * CASA/Rk

mortality/aging

mortality/aging ( J:101752 )

N • Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice

premature death ( J:101752 )

• exhibit a significant decrease in survival than wildtype

hematopoietic system

hematopoietic system phenotype ( J:101752 )

N • Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice

abnormal erythrocyte morphology ( J:101752 )

• peripheral blood smear shows schistocytes and fragmented red blood cells

thrombocytopenia ( J:101752 )

• decrease in average platelet count in peripheral blood

abnormal platelet physiology ( J:101752 )

• platelets show significantly prolonged adhesion to endothelial cells

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:101752 )

N • Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice

abnormal platelet physiology ( J:101752 )

• platelets show significantly prolonged adhesion to endothelial cells

increased circulating von Willebrand factor level ( J:101752 )

• 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)

abnormal thrombosis ( J:101752 )

• exhibit widespread vWF-rich and fibrin-poor hyaline thrombi in the small vessels of multiple organs

immune system

immune system phenotype ( J:101752 )

N • Background Sensitivity: associated phenotypes appear after crossing to CASA/Rk mice

increased susceptibility to bacterial infection ( 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

Genotype
MGI:4834630

cx4

• Allelic Composition: Adamts13^tm1Dgi/Adamts13^tm1Dgi; Vwf^tm1Wgr/Vwf^tm1Wgr
• Genetic Background: B6.129-Adamts13^tm1Dgi Vwf^tm1Wgr

hematopoietic system

hematopoietic system phenotype ( J:133469 )

N • mice exhibit the same LPS-induced thrombocytopenia as in Adamts13^tm1Dgi or Vwf^tm1Wgr homozygotes

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:133469 )

N • mice exhibit the same LPS-induced thrombocytopenia as in Adamts13^tm1Dgi or Vwf^tm1Wgr homozygotes

decreased susceptibility to induced thrombosis ( J:133469 )

• following FeCl3 injury, mice exhibit an increased time to clot formation and fail to completely occlude vessels unlike similarly treated wild-type mice

Genotype
MGI:4834624

cx5

• Allelic Composition: Adamts13^tm1Dgi/Adamts13^tm1Dgi; Vwf^tm1Wgr/Vwf^tm1Wgr
• Genetic Background: involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * CASA/Rk

mortality/aging

decreased susceptibility to xenobiotic induced morbidity/mortality ( J:133469 )

• in shigatoxin (Stx)-treated mice

homeostasis/metabolism

decreased susceptibility to xenobiotic induced morbidity/mortality ( J:133469 )

• in shigatoxin (Stx)-treated mice

decreased physiological sensitivity to xenobiotic ( J:133469 )

• shigatoxin (Stx)-treated mice exhibit less severe thrombocytopenia and reduced mortality compared with similarly treated Adamts13^tm1Dgi homozygotes

hematopoietic system

abnormal platelet morphology ( J:133469 )

• shigatoxin (Stx)-treated mice exhibit less severe thrombocytopenia and reduced mortality compared with similarly treated Adamts13^tm1Dgi homozygotes