Phenotypes Associated with This Genotype

Genotype
MGI:3694733

• Allelic Composition: Fas^lpr/Fas^lpr
• Genetic Background: involves: C3H * MRL/Mp

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

immune system

increased spleen weight ( J:34296 )

• at 2 months of age and on

increased susceptibility to type III hypersensitivity reaction ( J:34296 )

• beginning at 4 month of age

autoimmune response ( J:1060 )

• systemic autoimmune disease occurred at 2-3 months of age

• characterized by elevated serum immune complexes, cryoglobulins, and antinuclear antibodies

increased susceptibility to systemic lupus erythematosus ( J:34296 )

• beginning at 4 month of age

increased anti-nuclear antigen antibody level ( J:34296 )

• beginning at 4 month of age

hearing/vestibular/ear

abnormal stria vascularis morphology ( J:1060 , J:34296 )

• degeneration of the stria vascularis was seen starting at 2 month and progressed throughout the lifespan (J:1060)

• early edema of the stria occurred in the apex and progressed basalward (J:1060)

• by 10 months of age, stria vascularis area was smaller (J:1060)

• no degeneration of hair cells was seen at any age (J:1060)

• edematous areas in the stria vascularis primarily in the basal turns due to enlarged extracellular spaces filled with fluid (J:34296)

• all sensorineural elements, inner and outer hair cells and spiral ganglion neurons appeared normal even in the 8-10 month-old mice with significant threshold shifts (J:34296)

abnormal stria vascularis vasculature morphology ( J:34296 )

• the stria capillaries often were larger than normal, and the endothelial cells were occasionally swollen or thickened due to hypertrophy and not hyperplasia

increased or absent threshold for auditory brainstem response ( J:1060 , J:34296 )

• the ABR thresholds of the 10-month-old mutant mice were significantly higher than those of wild-type C3H/HeJ controls (J:1060)

• the 2- and 4-month-old mutant mice had normal auditory thresholds similar to control (J:34296)

• after onset of systemic autoimmune disease at 3-4 months of age, threshold at 6 months were significantly elevated at 24 and 32 kHz (J:34296)

• threshold continued to rise and by 8 months 16 kHz was elevated as well (J:34296)

• threshold at the low frequencies (4 and 8 kHz) did not change with progression of systemic disease (J:34296)

hematopoietic system

increased spleen weight ( J:34296 )

• at 2 months of age and on

cardiovascular system

abnormal stria vascularis vasculature morphology ( J:34296 )

• the stria capillaries often were larger than normal, and the endothelial cells were occasionally swollen or thickened due to hypertrophy and not hyperplasia

growth/size/body

increased spleen weight ( J:34296 )

• at 2 months of age and on