Mouse Genome Informatics
hm
    Cd40lgtm1Flv/Cd40lgtm1Flv
involves: 129S2/SvPas * C57BL/6
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• normal bone marrow monocyte/macrophage transition to giant multinucleate cells (osteoclasts) induced by Cd4+ T cells and reduced levels of IFN-gamma
• IFN-gamma treatment of cultured bone marrow macrophage suppresses osteoclast formation
• suppression of signaling via NF-kappaB ligand decreases osteoclast formation
• slightly decreased number of double positive thymocytes
• slight increase in the thymus and spleen although the ratio of CD4+ to Cd8+ cells in the spleen is normal
• no germinal center B cells in the spleens of immunized homozygotes but the architecture of the spleen is normal
• primary response to T cell dependent antigens is severely reduced
• secondary response to T cell dependent antigens is lost
• T cell independent antibody responses are unaffected
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• CD4+ T cells unable to drive isotype switching from IgM to IgG1 although IgM levels remain normal

skeleton
• normal bone marrow monocyte/macrophage transition to giant multinucleate cells (osteoclasts) induced by Cd4+ T cells and reduced levels of IFN-gamma
• IFN-gamma treatment of cultured bone marrow macrophage suppresses osteoclast formation
• suppression of signaling via NF-kappaB ligand decreases osteoclast formation

hematopoietic system
• normal bone marrow monocyte/macrophage transition to giant multinucleate cells (osteoclasts) induced by Cd4+ T cells and reduced levels of IFN-gamma
• IFN-gamma treatment of cultured bone marrow macrophage suppresses osteoclast formation
• suppression of signaling via NF-kappaB ligand decreases osteoclast formation
• slightly decreased number of double positive thymocytes
• slight increase in the thymus and spleen although the ratio of CD4+ to Cd8+ cells in the spleen is normal
• no germinal center B cells in the spleens of immunized homozygotes but the architecture of the spleen is normal
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• level is profoundly reduced in older animals, but is normal at 6 weeks of age
• CD4+ T cells unable to drive isotype switching from IgM to IgG1 although IgM levels remain normal

cellular
• normal bone marrow monocyte/macrophage transition to giant multinucleate cells (osteoclasts) induced by Cd4+ T cells and reduced levels of IFN-gamma
• IFN-gamma treatment of cultured bone marrow macrophage suppresses osteoclast formation
• suppression of signaling via NF-kappaB ligand decreases osteoclast formation

Mouse Models of Human Disease
OMIM IDRef(s)
NOT Immunodeficiency with Hyper-IgM, Type 1; HIGM1 308230 J:25010