Phenotypes Associated with This Genotype

Genotype
MGI:2449985

• Allelic Composition: Fas^lpr-cg/Fas^lpr-cg
• Genetic Background: CBA/KlJms-Fas^lpr-cg/J

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

mortality/aging

premature death ( J:24805 )

• earliest deaths at 19 and 29 weeks of age

• survival rate at 1 year is 61.1% in males and 46.2% in females

immune system

abnormal T cell differentiation ( J:24805 )

• anomalous differentiation of T cells, with anomalous expression of surface antigens

increased immunoglobulin level ( J:24805 )

increased IgG level ( J:24805 , J:81770 )

• five times higher than in controls (J:81770)

increased IgM level ( J:24805 , J:81770 )

• two times higher than in controls (J:81770)

abnormal lymph organ size ( J:24805 )

enlarged spleen ( J:24805 )

• palpable by 3 months of age

increased spleen weight ( J:81770 )

• spleens larger in females than males

• spleen weights increase up to 6 months of age and remain at a plateau thereafter

enlarged lymph nodes ( J:24805 , J:81770 )

• enlargement of the superficial lymph nodes starts at about 2.5 months of age, with cervical preceding inguinal lymph nodes (J:24805)

• superficial lymph nodes are more than 5 and 15 times heavier than controls at 3 and 5 months of age, respectively (J:24805)

• mesenteric lymph nodes are normal in size (J:24805)

abnormal hypersensitivity reaction ( J:24805 , J:81770 )

• lymphocyte infiltration in liver, lungs, spleen, and kidneys (J:24805)

autoimmune response ( J:24805 , J:81770 )

increased autoantibody level ( J:81770 )

increased anti-nuclear antigen antibody level ( J:24805 )

• elevated levels of autoantibodies to nuclear antigens including anti-DNA

hematopoietic system

enlarged spleen ( J:24805 )

• palpable by 3 months of age

increased spleen weight ( J:81770 )

• spleens larger in females than males

• spleen weights increase up to 6 months of age and remain at a plateau thereafter

abnormal T cell differentiation ( J:24805 )

• anomalous differentiation of T cells, with anomalous expression of surface antigens

increased immunoglobulin level ( J:24805 )

increased IgG level ( J:24805 , J:81770 )

• five times higher than in controls (J:81770)

increased IgM level ( J:24805 , J:81770 )

• two times higher than in controls (J:81770)

growth/size/body

enlarged spleen ( J:24805 )

• palpable by 3 months of age

increased spleen weight ( J:81770 )

• spleens larger in females than males

• spleen weights increase up to 6 months of age and remain at a plateau thereafter

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autoimmune lymphoproliferative syndrome		DOID:6688	OMIM:601859	J:24805 , J:81770