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Phenotypes Associated with This Genotype
Genotype
MGI:6405401
Allelic
Composition
Tardbptm2.1Neas/Tardbptm2.1Neas
Genetic
Background
B6(C3)-Tardbptm2.1Neas
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tardbptm2.1Neas mutation (0 available); any Tardbp mutation (44 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• no TDP-43 aggregation in ChAT+ neurons is seen in the lumbar spinal cord
• 2 year old mice show microgliosis in the lumbar spinal cord
• 2 year old mice show astrocytosis in the lumbar spinal cord
• modest, but significant, tibialis anterior muscle denervation is seen at 1.5 years of age
• greater denervation of the tibialis anterior muscle is seen at around 2.5 years of age, with asymmetric innervation of the tibialis anterior muscle and mice with the most denervation also showing the highest degree of asymmetry
• less denervation is seen in the soleus muscle than tibialis anterior muscle and no significant denervation of the gastrocnemius muscle is seen
• motor axon withdrawal precedes motoneuron loss
• no motor neuron loss is seen in the L4-5 spinal cord except for one mouse that showed 25% L4-5 motoneuron loss; this mouse has the largest degree of denervation of tibialis anterior muscle
• motoneuron loss in the one mouse is restricted to large-diameter motoneurons, indicating selective loss of alpha-motoneurons

immune system
• 2 year old mice show microgliosis in the lumbar spinal cord

hematopoietic system
• 2 year old mice show microgliosis in the lumbar spinal cord

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:283769


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
02/23/2021
MGI 6.16
The Jackson Laboratory