Phenotypes Associated with This Genotype

Genotype
MGI:3769009

• Allelic Composition: Entpd1^tm1Rdr/Entpd1^tm1Rdr
• Genetic Background: involves: 129S2/SvPas

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

cardiovascular system

cardiac fibrosis ( J:57505 )

abnormal blood flow velocity ( J:57505 )

• terminal erythrocyte velocity is increased (17.1+/-5.2 compared to 3.3+/-3.3 mm per second in wild-type mice)

abnormal vascular endothelial cell physiology ( J:57505 )

• ATPase and ADPase activities are substantially decreased compared to in wild-type mice

hematopoietic system

thrombocytopenia ( J:57505 )

• platelet count is 20% lower than in wild-type mice (538+/-78x10³ per mm³ compared to 740+/-81x10³ per mm³ in wild-type mice)

spleen fibrosis ( J:57505 )

abnormal neutrophil physiology ( J:129091 )

• polymorphonuclear (PMN) cells fail to convert etheno-AP into etheno-AMP as do wild-type PMNs

• following activation, the supernatant surrounding PMNs accumulate 1.6+/-0.09-fold more ATP than from wild-type PMNs

• unactivated PMN supernatant accumulates more ATP than wild-type

decreased platelet aggregation ( J:57505 )

• in vitro, platelets fail to aggregate in response to ADP, collagen or low doses of thrombin

• however, treatment with a soluble potato ATPase restores aggregation response

homeostasis/metabolism

decreased platelet aggregation ( J:57505 )

• in vitro, platelets fail to aggregate in response to ADP, collagen or low doses of thrombin

• however, treatment with a soluble potato ATPase restores aggregation response

increased bleeding time ( J:57505 )

• bleed time is increased to greater than 20 minutes in 4 mice and greater than 7 minutes in 7 mice compared to 1.0+/-0.2 minutes in heterozygous mice

• treatment with ferric chloride revealed a delay in platelet plug formation with no thrombi formation after 20 minutes compared to 100% in wild-type mice

immune system

spleen fibrosis ( J:57505 )

abnormal neutrophil physiology ( J:129091 )

• polymorphonuclear (PMN) cells fail to convert etheno-AP into etheno-AMP as do wild-type PMNs

• following activation, the supernatant surrounding PMNs accumulate 1.6+/-0.09-fold more ATP than from wild-type PMNs

• unactivated PMN supernatant accumulates more ATP than wild-type

respiratory system

pulmonary fibrosis ( J:57505 )

• fibrin deposits are present in the lungs and worsen after thrombogenic challenge

liver/biliary system

liver fibrosis ( J:57505 )

renal/urinary system

renal fibrosis ( J:57505 )

nervous system

abnormal brain morphology ( J:57505 )

• mice exhibit increased fibrin deposits in the brain