hematopoietic system
• neutrophils from homozygous mutant mice exhibit strong defects in Rap1 and beta1 and beta2 integrin activation while maintaining normal calcium flux, degranulation, and ROS generation
|
• neutrophils from homozygous mutant mice failed to adhere firmly to stimulated venules and to migrate into sites of inflammation
|
• platelets are unable to form stable thrombi when stimulated by collagen under flow conditions and mutants are resistant to collagen-induced thrombi mediated mortali
|
• aggregation of platelets in response to calcium ionophore, collagen, ADP and a thromboxane A analog is impaired
(J:92864)
• deficiency caused complete inhibition of arterial thrombus formation in mice
(J:122618)
|
immune system
• neutrophils from homozygous mutant mice exhibit strong defects in Rap1 and beta1 and beta2 integrin activation while maintaining normal calcium flux, degranulation, and ROS generation
|
• neutrophils from homozygous mutant mice failed to adhere firmly to stimulated venules and to migrate into sites of inflammation
|
homeostasis/metabolism
• platelets are unable to form stable thrombi when stimulated by collagen under flow conditions and mutants are resistant to collagen-induced thrombi mediated mortali
|
• aggregation of platelets in response to calcium ionophore, collagen, ADP and a thromboxane A analog is impaired
(J:92864)
• deficiency caused complete inhibition of arterial thrombus formation in mice
(J:122618)
|
• after tail transection mutants bleed for at least 30 minutes compared to less than 8 minutes for wild-type mice
• mutants do not develop spontaneous hemorrhage or anemia
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
leukocyte adhesion deficiency 3 | DOID:0110912 |
OMIM:612840 |
J:122618 |