Phenotypes Associated with This Genotype

Genotype
MGI:4360203

• Allelic Composition: Fas^lpr/Fas^lpr; Fcer1g^tm1Tks/Fcer1g^tm1Tks
• Genetic Background: MRL.Cg-Fcer1g^tm1Tks Fas^lpr

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

mortality/aging

premature death ( J:106188 )

• all mice diet of renal failure by 65 weeks of age with a 50% survival rate at a median age of 32-34 weeks similar to MRL-Fas^lpr mice

immune system

immune system phenotype ( J:106188 )

N • MRL-Fas mice homozygous for the Fcer1g^tm1Tks allele still develop glomerulonephritis in a similar manner as MRL-Fas^lpr mice carrying the wild-type Fcer1g^tm1Tks allele

arteritis ( J:106188 )

• necrotizing vasculitis with mononuclear cell infiltration, vessel wall destruction, and occasional thrombosis are observed in the interlobular arteries of the kidney

• necrotizing vasculitis of the pulmonary arteries, with mononuclear cell infiltration and vessel wall destruction occur

increased spleen weight ( J:106188 )

• spleen weight is 7- to 8-fold greater than C57BL/6 controls but is similar to MRL-Fas^lpr mice

increased double-negative T cell number ( J:106188 )

• a double-negative, B220⁺ T cell population accumulates in the spleen and lymph nodes similar to MRL-Fas^lpr mice

increased IgG level ( J:106188 )

• IgG levels are elevated compared to C57BL/6 controls but are similar to MRL-Fas^lpr mice

decreased susceptibility to type III hypersensitivity reaction ( J:106188 )

• extravasation does not occur in mice as occurs in controls when iv injected with OVA and intradermally injected with rabbit anti-OVA IgG

increased anti-double stranded DNA antibody level ( J:106188 )

• anti-DNA Iglevels are elevated compared to C57BL/6 controls but are similar to MRL-Fas^lpr mice

glomerulonephritis ( J:106188 )

• mice develop diffuse proliferative GN with mononuclear cell infiltration, mesangial and endothelial cell proliferation, and crescent formation after 16 weeks of age similar to MRL-Fas^lpr mice

• proteinuria, followed by renal failure, develops in all mice

renal/urinary system

abnormal glomerular capillary endothelium morphology ( J:106188 )

• endothelial cell proliferation is noted after 16 weeks of age

increased mesangial cell number ( J:106188 )

• mesangial cell proliferation is noted after 16 weeks of age

increased kidney cell proliferation ( J:106188 )

• mesangial and endothelial cell proliferation after 16 weeks of age

increased urine protein level ( J:106188 )

• urinary protein concentrations increase with age similar to MRL-Fas^lpr mice

glomerulonephritis ( J:106188 )

• mice develop diffuse proliferative GN with mononuclear cell infiltration, mesangial and endothelial cell proliferation, and crescent formation after 16 weeks of age similar to MRL-Fas^lpr mice

• proteinuria, followed by renal failure, develops in all mice

glomerular crescent ( J:106188 )

• crescent formation is noted after 16 weeks of age

kidney failure ( J:106188 )

• mice die of renal failure by 65 weeks of age similar to MRL-Fas^lpr mice

homeostasis/metabolism

increased urine protein level ( J:106188 )

• urinary protein concentrations increase with age similar to MRL-Fas^lpr mice

cardiovascular system

abnormal glomerular capillary endothelium morphology ( J:106188 )

• endothelial cell proliferation is noted after 16 weeks of age

vascular inflammation ( J:106188 )

• various forms of medium and small vessel vasculitis occur similar to MRL-Fas^lpr mice

arteritis ( J:106188 )

• necrotizing vasculitis with mononuclear cell infiltration, vessel wall destruction, and occasional thrombosis are observed in the interlobular arteries of the kidney

• necrotizing vasculitis of the pulmonary arteries, with mononuclear cell infiltration and vessel wall destruction occur

hematopoietic system

increased spleen weight ( J:106188 )

• spleen weight is 7- to 8-fold greater than C57BL/6 controls but is similar to MRL-Fas^lpr mice

increased double-negative T cell number ( J:106188 )

• a double-negative, B220⁺ T cell population accumulates in the spleen and lymph nodes similar to MRL-Fas^lpr mice

increased IgG level ( J:106188 )

• IgG levels are elevated compared to C57BL/6 controls but are similar to MRL-Fas^lpr mice

cellular

increased mesangial cell number ( J:106188 )

• mesangial cell proliferation is noted after 16 weeks of age

increased kidney cell proliferation ( J:106188 )

• mesangial and endothelial cell proliferation after 16 weeks of age

growth/size/body

increased spleen weight ( J:106188 )

• spleen weight is 7- to 8-fold greater than C57BL/6 controls but is similar to MRL-Fas^lpr mice