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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-TARDBP)96Dwc
transgene insertion 96, Don W Cleveland
MGI:5463113
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(Prnp-TARDBP)96Dwc/0
Tg(Prnp-TARDBP*Q331K)31Dwc/0
involves: C3H * C57BL/6 MGI:5828862
tg2
Tg(Prnp-TARDBP)96Dwc/0 involves: C3H * C57BL/6 MGI:5828861
tg3
Tg(Prnp-TARDBP)96Dwc/? involves: C57BL/6 * C3H MGI:5463235


Genotype
MGI:5828862
cx1
Allelic
Composition
Tg(Prnp-TARDBP)96Dwc/0
Tg(Prnp-TARDBP*Q331K)31Dwc/0
Genetic
Background
involves: C3H * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP)96Dwc mutation (1 available)
Tg(Prnp-TARDBP*Q331K)31Dwc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V

nervous system
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V
• loss of alpha motor neurons in layer V region of the cortex
• 46% loss of large caliber alpha-motor axons in L5 nerve root at 8 weeks of age
• decreased numbers of pyramidal neurons in layer V
• astrogliosis is observed in layer V of cortex of young animals
• mild increase in CD68+ microgliosis in layer V
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
• astrogliosis is observed in layer V of cortex of young animals
• astrogliosis is observed in anterior horn of spinal cord in young mice
• 46% loss of large caliber alpha-motor axons in L5 nerve root at 8 weeks of age
• axon degeneration is characterized by vacuolization and myelin defects
• 70% loss of alpha motor neurons in anterior horn of lumbar spinal cord
• loss of alpha motor neurons in layer V region of the cortex
• decreased numbers of pyramidal neurons in layer V
• decreased numbers of pyramidal neurons in layer V
• 60% reduction in intact neuromuscular junctions in 8 week old mice
• reduction in NMJ area in 8 week old mice
• frequent globular cytoplasmic inclusions are observed in spinal cord at 8 weeks of age
• a small number of ubiquitin and p62+ inclusions are observed in spinal cord of 8 week old mice
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
• multiple small globular inclusions in neuropil of ventral horns
• nuclear aggregation of endogenous TDP-43 in 8 week old mice
• astrogliosis and mild gliosis in anterior horn of spinal cord in young mice
• frequent globular cytoplasmic inclusions are observed in spinal cord at 8 weeks of age
• multiple small globular inclusions in neuropil of ventral horns
• moderate gliosis is observed in white matter tracts
• 70% loss of alpha motor neurons in anterior horn of lumbar spinal cord
• progressive motor and cortical neuron degeneration

immune system
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V

mortality/aging
• average survival time is 64 +/- 4 days

muscle
• muscle fiber atrophy

behavior/neurological
• failure to splay limbs normally when lifted by tail
• tremor observed beginning at 3 weeks of age
• rapid decline in motor function from 3 weeks of age
• decline in performance on rotarod beginning at 5 weeks of age as compared to controls
• severely stilted gait
• progressive, severe hind-limb paralysis beginning at 5-6 weeks of age
• mice lack the ability to raise pelvis from ground

digestive/alimentary system
N
• no intestinal blockage or megacolon is observed

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:239141




Genotype
MGI:5828861
tg2
Allelic
Composition
Tg(Prnp-TARDBP)96Dwc/0
Genetic
Background
involves: C3H * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP)96Dwc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• no significant motor dysfunction is observed in mice up to 24 months of age
• cytoplasmic and intranuclear inclusions are not observed




Genotype
MGI:5463235
tg3
Allelic
Composition
Tg(Prnp-TARDBP)96Dwc/?
Genetic
Background
involves: C57BL/6 * C3H
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP)96Dwc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype





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
10/08/2019
MGI 6.14
The Jackson Laboratory