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Phenotypes Associated with This Genotype
involves: C57BL/6 * SJL
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phenotype observed in females
phenotype observed in males
N normal phenotype
• at 24 months of age, 72% of mutants exhibit limb twitch compared to 18% of wild-type mice
• mutants develop progressive motor weakness
• impaired ability to stand on a slanted surface
• dystonic movement of the hindlimbs or both hindlimbs and forelimbs when lifted by tails

• dystonic movement of the hindlimbs or both hindlimbs and forelimbs when lifted by tails

nervous system
• mutants develop tau inclusions in both neurons and glia of spinal cord and ventral roots
• accumulating tau proteins in oligodendrocytes progressively form aggregates that resemble coiled bodies in human tauopathies
• tau proteins are located in the cytoplasm of Schwann cells
• tau accumulation is also seen in the molecular layer of the cerebellum, mostly in vertically oriented redial glial fibers
• progressive accumulation of tau in astrocytes and oligodendrocytes is associated with degeneration of these cells
• mutants develop tau-positive astrocytic plaque-like lesions at 12 months of age, with a peak in number at 15 months and a decline thereafter, indicating a loss of astrocytes with age
• reduction in oligodendrocytes in the lumber spinal cord at 6 months of age, indicating degeneration of oligodendrocytes
• degeneration of Schwann cells
• 25 month old mutants exhibit myelin disruption in the spinal cord and ventral roots such as thinning of myelin sheaths in the ventral roots, disorganization of the compact myelin in the spinal cord, detachment of myelin layers in old mutants, and structural disorganization of myelin
• mutants exhibit an age-related reduction in neurons in the anterior horn of the lumbar spinal cord

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
frontotemporal dementia DOID:9255 OMIM:600274

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
MGI 6.13
The Jackson Laboratory