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Phenotypes Associated with This Genotype
involves: C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Camk2a-tTA)1Mmay mutation (8 available)
Tg(tetO-TARDBP*)4Vle mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• about 50% loss of dentate gyrus neurons at 1 month after Dox removal
• massive gliosis in cortical and hippocampal regions following Dox removal
• gliosis in the corticospinal tract, including the striatum, cerebral peduncles, medullary pyramids, and cervical spinal cord, following Dox removal
• selective loss of corticospinal tract axons in cervical spinal cord associated with gliosis following Dox removal
• however, lower motor neuron loss is not observed
• mutants rarely show accumulation of hyperphosphorylated, ubiquitinated cytoplasmic aggregates of TARDBP in neurons following Dox removal (around 1% of neurons showing aggregates)
• mutants switched to a doxycycline (dox)-free diet at 28 days of age show progressive neurodegeneration; neuron loss is particularly evident in the deep neocortical layers and in the dentate gyrus but is rarely seen in the hippocampal CA1 subfield and the olfactory bulb
• selective loss of corticospinal tract axons in cervical spinal cord following Dox removal

• abnormal limb clasping as early as 1 week after dox removal that continues throughout life until sacrifice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069

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
MGI 6.17
The Jackson Laboratory