Mouse Genome Informatics
hm
    Faslgld/Faslgld
C3H/HeJ-Faslgld/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
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 (J:120650)
• 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 (J:7306)

hematopoietic system
• evident in 40% of animals autopsied when moribund
• 2-fold greater than controls
• 4-fold greater than controls (J:7306)
• 5-fold increase in peripheral blood lymphocytes (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)
• evident after 13 weeks of age (J:7306)
• 4-fold enlargement compared to controls (J:29572)
• 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
• ~25% of those autopsied when moribund showed marked subcutaneous edema

immune system
• 2-fold greater than controls
• 4-fold greater than controls (J:7306)
• 5-fold increase in peripheral blood lymphocytes (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)
• evident after 13 weeks of age (J:7306)
• 4-fold enlargement compared to controls (J:29572)
• 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 (J:7306)

respiratory system
• lung inflammation resembling interstitial pneumonitis evident in virtually all animals autopsied when moribund

tumorigenesis
N
• regression of transplanted SCCVII tumors by gene therapy treatment with Il12b is normal (J:108303)

integument
• ~25% of those autopsied when moribund showed marked subcutaneous edema

Mouse Models of Human Disease
OMIM IDRef(s)
Autoimmune Lymphoproliferative Syndrome; ALPS 601859 J:7306 , J:29572