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Phenotypes Associated with This Genotype
Genotype
MGI:5788563
Allelic
Composition
Cln3tm1Nbm/Cln3tm1Nbm
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cln3tm1Nbm mutation (3 available); any Cln3 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• mutant bone marrow macrophages exhibit increased adhesiveness to CD11c ligands in vitro
• bone marrow macrophages stimulated with LPS exhibit decreased secretion of IFN-gamma, IL-1beta, IL-17, IL-12p70 and IL-9
• increase in surface and total protein levels of CD11c in antigen presenting cells (bone marrow macrophages, bone marrow dendritic cells, and peritoneal macrophages)
• increase in surface levels of CD11c on mononuclear phagocytes (CD14+ monocytes in the blood and F4/80+ macrophages in the spleen)
• decrease in surface levels of CD11b in bone marrow macrophages and dendritic cells and peritoneal macrophages
• mutant bone marrow dendritic cells exhibit increased adhesiveness to CD11c ligands in vitro

hematopoietic system
• mutant bone marrow macrophages exhibit increased adhesiveness to CD11c ligands in vitro
• bone marrow macrophages stimulated with LPS exhibit decreased secretion of IFN-gamma, IL-1beta, IL-17, IL-12p70 and IL-9

cellular
• mutant bone marrow dendritic cells and bone marrow marcrophages exhibit increased adhesiveness to CD11c ligands in vitro

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
neuronal ceroid lipofuscinosis 3 DOID:0110731 OMIM:204200
J:233445


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/16/2019
MGI 6.13
The Jackson Laboratory