Mouse Genome Informatics
hm
    Fastm1Osa/Fastm1Osa
involves: 129P2/OlaHsd * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Histopathology of Fas-deficient mice.

mortality/aging
• 50% dead at about 5 months of age

liver/biliary system
• hepatocyte nuclear areas are variable and larger
• significantly heavier at 12 weeks of age and becomes 50-60% heavier by 16 weeks of age
• number of hepatocytes per each liver lobe is increased

immune system
• 20x higher than in controls, attributable to increased lymphocyte numbers
• lymphocytosis; progressively accumulate abnromal T cells Thy1+ B220+ CD4- CD8 - (J:64296)
• increase in the number of normal B cells
• peripheral clonal deletion of mature T cells against a bacterial superantigen is impaired
• 10-100x higher serum levels of IgG1, IgG2a, IgG2b, and IgG3 at 16 weeks of age
• spleen becomes progressively larger (J:64296)
• lymph nodes become progressively larger (J:64296)
• occasional infiltration of lymphocytes into tissues such as the lungs and liver (J:64296)
• 50-500x higher levels of dsDNA and ssDNA autoantibodies at 16 weeks of age
• decreased survival after infection with Trypanosoma cruzi even though were able to clear parasites from blood and affected organs

hematopoietic system
• 20x higher than in controls, attributable to increased lymphocyte numbers
• lymphocytosis; progressively accumulate abnromal T cells Thy1+ B220+ CD4- CD8 - (J:64296)
• increase in the number of normal B cells
• peripheral clonal deletion of mature T cells against a bacterial superantigen is impaired
• spleen becomes progressively larger (J:64296)
• 10-100x higher serum levels of IgG1, IgG2a, IgG2b, and IgG3 at 16 weeks of age

Mouse Models of Human Disease
OMIM IDRef(s)
Autoimmune Lymphoproliferative Syndrome; ALPS 601859 J:32125