Phenotypes Associated with This Genotype

Genotype
MGI:4461233

• Allelic Composition: Tg(Igh-V-CD40lg)#Tsub/0
• Genetic Background: involves: C57BL/6

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

digestive/alimentary system

abnormal intestinal mucosa morphology ( J:89855 )

• epithelial hyperplasia with glandular elongation in whole colon and ileum

abnormal ileum crypts of Lieberkuhn morphology ( J:89855 )

• crypt hyperplasia within the ileum

abnormal colon morphology ( J:89855 )

• thicked wall

• mononuclear cells in colon significantly increased in 15-wk-old mice

abnormal colon goblet cell morphology ( J:89855 )

• goblet cell depletion in whole colon

megacolon ( J:89855 )

diarrhea ( J:89855 )

• 90% of mice at 20 weeks

colitis ( J:89855 )

• severe colitis in 15-wk-old mice, mostly B220+ cells and CD8+ cells

• extensive leukocytic infiltrates were seen in the lamina propria; and submucosa and, to a lesser degree, in the muscularis, serosa, and mesentery

growth/size/body

weight loss ( J:89855 )

• significant weight loss until 20 wk of age

enlarged spleen ( J:89855 )

hematopoietic system

increased splenocyte proliferation ( J:89855 )

• increased mononuclear proliferation in spleen

enlarged spleen ( J:89855 )

increased splenocyte number ( J:89855 )

• mononuclear cells in spleen significantly increased in 15-wk-old mice

increased IgG level ( J:89855 )

• significantly increased by 4 fold

increased IgM level ( J:89855 )

• significantly increased by 5 fold

immune system

increased splenocyte proliferation ( J:89855 )

• increased mononuclear proliferation in spleen

colitis ( J:89855 )

• severe colitis in 15-wk-old mice, mostly B220+ cells and CD8+ cells

• extensive leukocytic infiltrates were seen in the lamina propria; and submucosa and, to a lesser degree, in the muscularis, serosa, and mesentery

enlarged spleen ( J:89855 )

increased splenocyte number ( J:89855 )

• mononuclear cells in spleen significantly increased in 15-wk-old mice

increased IgG level ( J:89855 )

• significantly increased by 4 fold

increased IgM level ( J:89855 )

• significantly increased by 5 fold

abnormal bronchus-associated lymphoid tissue morphology ( J:89855 )

• hyperplastic bronchus-associated lymphoid tissue in lungs of half of the mice

enlarged lymph nodes ( J:89855 )

• enlarged mesenteric lymph nodes

increased interferon-gamma secretion ( J:89855 )

• by lamina propria CD4 T cells stimulated in culture

increased autoantibody level ( J:89855 )

• IgM autoantibodies that reacts with colon crypt epithelium

• IgG autoantibodies that show a strong reaction with surface epithelium of the colon

glomerulonephritis ( J:89855 )

• 50% of mice

endocrine/exocrine glands

abnormal colon goblet cell morphology ( J:89855 )

• goblet cell depletion in whole colon

abnormal ileum crypts of Lieberkuhn morphology ( J:89855 )

• crypt hyperplasia within the ileum

renal/urinary system

glomerulonephritis ( J:89855 )

• 50% of mice

cellular

abnormal colon goblet cell morphology ( J:89855 )

• goblet cell depletion in whole colon

increased splenocyte proliferation ( J:89855 )

• increased mononuclear proliferation in spleen

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
inflammatory bowel disease		DOID:0050589	OMIM:PS266600	J:89855