Phenotypes Associated with This Genotype

Genotype
MGI:8332036

• Allelic Composition: Braf^tm1Mmcm/Braf⁺; Lrig1^{tm1.1(cre/ERT2)Rjc}/Lrig1⁺
• Genetic Background: involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J

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

neoplasm

increased oral papilloma incidence ( J:300991 )

• mice develop oral tumors 12 weeks after a single injection of tamoxifen at 8 weeks of age; tumors are squamous papillomas

increased gastrointestinal stromal tumor incidence ( J:300991 )

• the outer longitudinal muscular layer is almost completely replaced by lesions with diffuse CD34 staining, indicating gastrointestinal stromal tumors (GIST) and active proliferation of the hyperplastic lesions is seen

• similar lesions are seen in the small intestine and colon mostly in the outer longitudinal muscular layer, although less prominently than in the stomach

behavior/neurological

abnormal food intake ( J:300991 )

• mice treated with tamoxifen are unable to eat due to development of oral tumors

cellular

interstitial cells of Cajal hyperplasia ( J:300991 )

• hyperplastic regions are devoid of smooth muscle actin and partially positive for KIT, resembling interstitial cells of Cajal (ICC) hyperplasia in the gastric wall

craniofacial

increased oral papilloma incidence ( J:300991 )

• mice develop oral tumors 12 weeks after a single injection of tamoxifen at 8 weeks of age; tumors are squamous papillomas

digestive/alimentary system

interstitial cells of Cajal hyperplasia ( J:300991 )

• hyperplastic regions are devoid of smooth muscle actin and partially positive for KIT, resembling interstitial cells of Cajal (ICC) hyperplasia in the gastric wall

abnormal stomach wall morphology ( J:300991 )

• 8-10 weeks after tamoxifen administration, mice show a thickened gastric wall

abnormal stomach non-glandular epithelium morphology ( J:300991 )

• squamous hyperplasia of the forestomach in tamoxifen-treated mice

abnormal stomach muscularis externa morphology ( J:300991 )

• the outer half of the muscularis propria is replaced by hyperplastic spindle-shaped cells with dysplastic features in tamoxifen-treated mice

increased gastrointestinal stromal tumor incidence ( J:300991 )

• the outer longitudinal muscular layer is almost completely replaced by lesions with diffuse CD34 staining, indicating gastrointestinal stromal tumors (GIST) and active proliferation of the hyperplastic lesions is seen

• similar lesions are seen in the small intestine and colon mostly in the outer longitudinal muscular layer, although less prominently than in the stomach

growth/size/body

increased oral papilloma incidence ( J:300991 )

• mice develop oral tumors 12 weeks after a single injection of tamoxifen at 8 weeks of age; tumors are squamous papillomas

muscle

abnormal stomach muscularis externa morphology ( J:300991 )

• the outer half of the muscularis propria is replaced by hyperplastic spindle-shaped cells with dysplastic features in tamoxifen-treated mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
gastrointestinal stromal tumor		DOID:9253	OMIM:606764	J:300991