mortality/aging
N |
• when placed under hyperoxic conditions for >5 days, mice do not show increased survival (resistance to hyperoxia) compared to wild-type mice
|
• males survived to mean age of 396 days, females to 369 days, controls survived until 688 days
(J:7306)
|
cardiovascular system
N |
• no defect detected: no vascular disease, including necrotizing arteritis or polyarteritis
|
hematopoietic system
• evident after 13 weeks of age
(J:7306)
• 4-fold enlargement compared to controls
(J:29572)
|
• abnormal cells populating lymph nodes during lymphoproliferation fail to show characteristics of immature or mature T cells:
(J:8267)
• express beta-chain of TCR
(J:8267)
• exhibit rearrangements of beta-chain genes
(J:8267)
• express TCR beta and alpha gene mRNA
(J:8267)
• are Thy-1+, Ly-1+, Ly-2-, L3T4-, Ly-5(B220)+, Ly-6+, Ly-22+, Ly-24+, sIg-, ThB-, Ia-, HSA-/+, and PC.1+
(J:8267)
• bind at high levels lectins that normally bind preferentially to B cells
(J:8267)
• did not proliferate or generate CTL in response to stimulation with alloantigens
(J:8267)
• cells stimulated with Con A failed to produce IL-2
(J:8267)
• abnormal cells populating lymph nodes during lymphoproliferation fail to show characteristics of immature or mature T cells:
(J:12002)
• express beta-chain of TCR
(J:12002)
• exhibit rearrangements of beta-chain genes
(J:12002)
• express TCR beta and alpha gene mRNA
(J:12002)
• are Thy-1+, Ly-1+, Ly-2-, L3T4-, Ly-5(B220)+, Ly-6+, Ly-22+, Ly-24+, sIg-, ThB-, Ia-, HSA-/+, and PC.1+
(J:12002)
• bind at high levels lectins that normally bind preferentially to B cells
(J:12002)
• did not proliferate or generate CTL in response to stimulation with alloantigens
(J:12002)
• cells stimulated with Con A failed to produce IL-2
(J:12002)
|
• 36 to 15%
|
• 2-fold greater than controls
|
• 4-fold greater than controls
(J:7306)
• 5-fold increase in peripheral blood lymphocytes
(J:29572)
|
• 59 to 68%
|
• development of broad-based hypergammaglobulinemia
(J:7306)
• developed broad-based hypergammaglobulinemia
(J:29572)
|
• 10-fold
(J:29572)
|
• 10-fold IgG2a
• 3- to 6-fold IgG1 and IgG2b
|
• in Fas-dependent lysis assays, but not allogeneic targets
|
homeostasis/metabolism
skin edema
(
J:7306
)
• ~25% of those autopsied when moribund showed marked subcutaneous edema
|
immune system
• evident after 13 weeks of age
(J:7306)
• 4-fold enlargement compared to controls
(J:29572)
|
• abnormal cells populating lymph nodes during lymphoproliferation fail to show characteristics of immature or mature T cells:
(J:8267)
• express beta-chain of TCR
(J:8267)
• exhibit rearrangements of beta-chain genes
(J:8267)
• express TCR beta and alpha gene mRNA
(J:8267)
• are Thy-1+, Ly-1+, Ly-2-, L3T4-, Ly-5(B220)+, Ly-6+, Ly-22+, Ly-24+, sIg-, ThB-, Ia-, HSA-/+, and PC.1+
(J:8267)
• bind at high levels lectins that normally bind preferentially to B cells
(J:8267)
• did not proliferate or generate CTL in response to stimulation with alloantigens
(J:8267)
• cells stimulated with Con A failed to produce IL-2
(J:8267)
• are Thy-1+, Ly-1+, Ly-2-, L3T4-, Ly-5(B220)+, Ly-6+, Ly-22+, Ly-24+, sIg-, ThB-, Ia-, HSA-/+, and PC.1+
(J:12002)
• abnormal cells populating lymph nodes during lymphoproliferation fail to show characteristics of immature or mature T cells:
(J:12002)
• express beta-chain of TCR
(J:12002)
• exhibit rearrangements of beta-chain genes
(J:12002)
• express TCR beta and alpha gene mRNA
(J:12002)
• bind at high levels lectins that normally bind preferentially to B cells
(J:12002)
• did not proliferate or generate CTL in response to stimulation with alloantigens
(J:12002)
• cells stimulated with Con A failed to produce IL-2
(J:12002)
|
• 36 to 15%
|
• 2-fold greater than controls
|
• 4-fold greater than controls
(J:7306)
• 5-fold increase in peripheral blood lymphocytes
(J:29572)
|
• 59 to 68%
|
• development of broad-based hypergammaglobulinemia
(J:7306)
• developed broad-based hypergammaglobulinemia
(J:29572)
|
• 10-fold
(J:29572)
|
• 10-fold IgG2a
• 3- to 6-fold IgG1 and IgG2b
|
• in Fas-dependent lysis assays, but not allogeneic targets
|
• enlarged peripheral lymph nodes evident at 13 weeks of age, abdominal evident shortly thereafter
(J:7306)
• evidence of chronic inflammation at autopsy, with proliferation of lymphocytes and admixtures of histiocytes and plasma cells observed, fibrosis and multinucleated giant cells also frequently observed
(J:7306)
• 50-fold heavier at 20 weeks of age than controls
(J:29572)
|
• thymocyte-binding autoantibody present
|
• high titers of antinuclear autoantibodies evident by 16 weeks of age in all mice assayed
(J:7306)
• high titers of antinuclear autoantibodies evident at 14 weeks of age
(J:29572)
|
• high concentrations of anti-dsDNA autoantibodies present
|
• lung inflammation resembling interstitial pneumonitis evident in virtually all animals autopsied when moribund
|
renal/urinary system
N |
• despite glomerular deposition of immune complexes, no, or very little, glomerulonephritis was observed
|
respiratory system
• lung inflammation resembling interstitial pneumonitis evident in virtually all animals autopsied when moribund
|
neoplasm
N |
• regression of transplanted SCCVII tumors by gene therapy treatment with Il12b is normal
|
integument
skin edema
(
J:7306
)
• ~25% of those autopsied when moribund showed marked subcutaneous edema
|
growth/size/body
• evident after 13 weeks of age
(J:7306)
• 4-fold enlargement compared to controls
(J:29572)
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
autoimmune lymphoproliferative syndrome | DOID:6688 |
OMIM:601859 |
J:7306 , J:29572 |