immune system
N |
• mice exhibit normal NK cell accessory cells
|
• NK cell maturation is blocked at the double positive stage unlike in wild-type mice
|
• mice exhibit an accumulation of atypical myeloid precursors in the bone marrow compared with wild-type mice
|
• in the blood, spleen, bone marrow, liver, lungs and lymph nodes
|
• hyporesponsive towards YCA-1 target cells in vitro and MHC-I-deficient splenocytes in vivo
• hyporesponsive towards stimulation of NK1.1-, NKp46- or NKG2D- and Ly49D-activatng receptors
• transplant experiments confirm that NK cell hyporeactivity involves a cell-extrinsic factor
• NK cells exhibit higher rates of proliferation and poorer survival compared with wild-type cells
• however, responsiveness to PMA and ionomycin stimulation is normal and NK cells regain full reactivity in a wild-type environment
|
• after stimulation with YAC-1 target cells
|
hematopoietic system
• NK cell maturation is blocked at the double positive stage unlike in wild-type mice
|
• mice exhibit an accumulation of atypical myeloid precursors in the bone marrow compared with wild-type mice
|
• in the blood, spleen, bone marrow, liver, lungs and lymph nodes
|
• hyporesponsive towards YCA-1 target cells in vitro and MHC-I-deficient splenocytes in vivo
• hyporesponsive towards stimulation of NK1.1-, NKp46- or NKG2D- and Ly49D-activatng receptors
• transplant experiments confirm that NK cell hyporeactivity involves a cell-extrinsic factor
• NK cells exhibit higher rates of proliferation and poorer survival compared with wild-type cells
• however, responsiveness to PMA and ionomycin stimulation is normal and NK cells regain full reactivity in a wild-type environment
|
• after stimulation with YAC-1 target cells
|
cellular
• after stimulation with YAC-1 target cells
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
severe congenital neutropenia | DOID:0050590 |
OMIM:PS202700 |
J:182679 |