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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-TARDBP*Q331K)103Dwc
transgene insertion 103, Don W Cleveland
MGI:5463119
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-TARDBP*Q331K)103Dwc/0 B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J MGI:6095193
tg2
Tg(Prnp-TARDBP*Q331K)103Dwc/? involves: C57BL/6 * C3H MGI:5463233


Genotype
MGI:6095193
tg1
Allelic
Composition
Tg(Prnp-TARDBP*Q331K)103Dwc/0
Genetic
Background
B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*Q331K)103Dwc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• induction of glutathione levels in response to sodium arsenite is impaired in primary astrocyte cultures and mice show an increase in transcripts of the antioxidant genes Hmox1 and Nqo1in the spinal cord, indicating elevated oxidative stress and impaired antioxidant response

nervous system
• loss of insoluble heterogeneous nuclear ribonucleoprotein K in the brain

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:242322




Genotype
MGI:5463233
tg2
Allelic
Composition
Tg(Prnp-TARDBP*Q331K)103Dwc/?
Genetic
Background
involves: C57BL/6 * C3H
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*Q331K)103Dwc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• hind limb clasping develops by 3 months of age
• develop tremors around 3 months of age
• motor deficits appear by 3 months when tested on a rotarod
• in hind limbs

muscle
• regions of damaged muscle fibers and other areas of regeneration
• spontaneous gastrocnemius muscle fibrillations
• reduced myogenic motor evoked potentials after stimulation of motor cortex
• regions of damaged muscle fibers and other areas of regeneration

nervous system
• age dependent decrease in motor neuron number of about 35%
• most significant reductions in large caliber alpha motor axons at 10-12 months
• reduced number of gastrocnemius neuromuscular junction endplates
• no differences from controls recorded at the thoracic T12 segment after stimulation of motor cortex





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
01/14/2020
MGI 6.14
The Jackson Laboratory