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Phenotypes Associated with This Genotype
Genotype
MGI:6192275
Allelic
Composition
Braftm1Mmcm/Braftm1Mmcm
Tg(Itgax-cre)1-1Reiz/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Braftm1Mmcm mutation (3 available); any Braf mutation (44 available)
Tg(Itgax-cre)1-1Reiz mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
liver/biliary system
• severe hepatosplenomegaly

mortality/aging
• decrease in lifespan

immune system
• severe hepatosplenomegaly
• lesions are associated with massive increase of MHC II+CD11c+ dendritic cells
• expansion of blood circulating dendritic cell precursors and blood circulating dendritic cells
• histiocytic infiltrates in the skin, liver, spleen, and lungs by 8 weeks of age, resulting in a broad destruction of tissue architecture by 16 weeks of age
• histiocytic lesions exhibit classical granulomatous organization, including multinucleated giant cell formation
• granuloma lesions contain massive CD11c+ langerin+ infiltrates, a large number of macrophages, NK cells, B cells, and T cells, with a specific accumulation of regulatory T cells
• local fibrotic response is seen within the granulomas and surrounding stroma
• mice rapidly develop an aggressive Langerhans-cells histiocytosis-like disease with 100% penetrance
• granuloma lesions are associated with an increase in several chemokines and cytokines (Ccl2, Ccl15, Il6, Il10, IFN-gamma, and TGF-beta) indicative of a local cytokine storm

hematopoietic system
• severe hepatosplenomegaly
• severe anemia
• lesions are associated with massive increase of MHC II+CD11c+ dendritic cells
• expansion of blood circulating dendritic cell precursors and blood circulating dendritic cells

growth/size/body
• severe hepatosplenomegaly

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Langerhans-cell histiocytosis DOID:2571 OMIM:246400
OMIM:604856
J:211690


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
01/12/2022
MGI 6.17
The Jackson Laboratory