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Phenotypes Associated with This Genotype
Genotype
MGI:6314154
Allelic
Composition
Grntm2.1Far/Grntm2.1Far
Genetic
Background
involves: 129S4/SvJae * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grntm2.1Far mutation (1 available); any Grn mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Microgliosis, thalamic neuronal TDP-43+ cytoplasmic inclusions, reduced synaptic density, lipofuscinosis, and skin lesions due to excessive grooming in Grntm2.1Far/Grntm2.1Far mice

mortality/aging
• 30% lower median survival of 575 days compared to 816 days in wild-type mice
• 30% lower median survival of 575 days compared to 816 days in wild-type mice

behavior/neurological
• mice exhibit compulsive grooming

hematopoietic system
• mice exhibit age-dependent microgliosis
• bone marrow-derived macrophages exhibit an exaggerated inflammatory response after treatment with lipopolysaccharide (LPS) and IFN-gamma

immune system
• mice exhibit age-dependent microgliosis
• bone marrow-derived macrophages exhibit an exaggerated inflammatory response after treatment with lipopolysaccharide (LPS) and IFN-gamma

integument
• compulsive grooming leads to development of skin lesions over time

nervous system
• mice exhibit age-dependent microgliosis
• mice exhibit increased levels of total and phosphorylated TDP-43 in the cytoplasm of thalamic neurons
• mice exhibit an age-dependent reduction in synapse density
• mice exhibit increased levels of total and phosphorylated TDP-43 in the cytoplasm of thalamic neurons

pigmentation
• mice exhibit an increase in levels of lipofuscin in the brain indicating lysosomal dysfunction


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
07/09/2019
MGI 6.14
The Jackson Laboratory