Phenotypes Associated with This Genotype

Genotype
MGI:3799686

• Allelic Composition: Fas^lpr/Fas^lpr
• Genetic Background: MRL/MpJ-Fas^lpr

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:125114 , J:137066 )

• animals start to die at 4.5 months, with >50% mortality observed at 7 months (J:125114)

• 50% mortality by 20 weeks; <40% survival beyond 40 weeks (J:137066)

hematopoietic system

abnormal leukocyte adhesion ( J:126009 )

• significantly enhanced at 12 and 16 weeks

impaired leukocyte tethering or rolling ( J:126009 )

• rolling is dramatically reduced, but not eliminated, in mutants compared to controls

• in mice chronically treated with anti-E-selectin antibodies, rolling is completely eliminated

enlarged spleen ( J:137066 )

• severe

abnormal leukocyte morphology ( J:126009 )

• 46% of venules display leukocytes adjacent to endothelium, compared to only 145 in controls; in mutants and controls, 60-70% of these cells are mononuclear

increased double-negative T cell number ( J:137066 )

• significantly increased relative to controls

decreased B cell number ( J:137066 )

decreased CD4-positive, alpha-beta T cell number ( J:137066 )

• reduced compared to wild-type MRL animals

decreased CD8-positive, alpha-beta T cell number ( J:137066 )

• reduced compared to wild-type MRL animals

decreased activated T cell number ( J:137066 )

increased immunoglobulin level ( J:125114 )

• mice develop hypergammaglobulinemia

immune system

blood vessel inflammation ( J:137066 )

• mice develop systemic necrotizing arteritis of small- and medium-sized arteries; frequently observed in kidneys

abnormal leukocyte adhesion ( J:126009 )

• significantly enhanced at 12 and 16 weeks

impaired leukocyte tethering or rolling ( J:126009 )

• rolling is dramatically reduced, but not eliminated, in mutants compared to controls

• in mice chronically treated with anti-E-selectin antibodies, rolling is completely eliminated

enlarged spleen ( J:137066 )

• severe

abnormal leukocyte morphology ( J:126009 )

• 46% of venules display leukocytes adjacent to endothelium, compared to only 145 in controls; in mutants and controls, 60-70% of these cells are mononuclear

increased double-negative T cell number ( J:137066 )

• significantly increased relative to controls

decreased B cell number ( J:137066 )

decreased CD4-positive, alpha-beta T cell number ( J:137066 )

• reduced compared to wild-type MRL animals

decreased CD8-positive, alpha-beta T cell number ( J:137066 )

• reduced compared to wild-type MRL animals

decreased activated T cell number ( J:137066 )

increased immunoglobulin level ( J:125114 )

• mice develop hypergammaglobulinemia

enlarged lymph nodes ( J:137066 )

• severe

autoimmune response ( J:125114 )

• mice develop anti-nuclear antibodies (ie. anti-dsDNA, anti-ssDNA, etc)

increased autoantibody level ( J:137066 )

• IgG3 anti-IgG2a rheumatoid factor (RF) levels are much higher than wild-type controls

increased anti-nuclear antigen antibody level ( J:137066 )

• levels of anti-dsDNA and anti-chromatin autoantibodies are elevated compared to wild-type

glomerulonephritis ( J:125114 , J:132514 , J:137066 )

• mice show deposition of IgG or C3 in kidneys and inflammation (J:125114)

cardiovascular system

blood vessel inflammation ( J:137066 )

• mice develop systemic necrotizing arteritis of small- and medium-sized arteries; frequently observed in kidneys

renal/urinary system

glomerulonephritis ( J:125114 , J:132514 , J:137066 )

• mice show deposition of IgG or C3 in kidneys and inflammation (J:125114)

renal glomerular immunoglobulin deposits ( J:125114 )

cellular

abnormal leukocyte adhesion ( J:126009 )

• significantly enhanced at 12 and 16 weeks

impaired leukocyte tethering or rolling ( J:126009 )

• rolling is dramatically reduced, but not eliminated, in mutants compared to controls

• in mice chronically treated with anti-E-selectin antibodies, rolling is completely eliminated

growth/size/body

enlarged spleen ( J:137066 )

• severe