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Phenotypes Associated with This Genotype
Genotype
MGI:5308055
Allelic
Composition
Tg(Prnp-TARDBP)3cPtrc/Tg(Prnp-TARDBP)3cPtrc
Genetic
Background
C57BL/6-Tg(Prnp-TARDBP)3cPtrc
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP)3cPtrc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants become moribound between 1 and 2 months of age (J:163231)
• mutants become moribound between 1 and 2 months of age (J:163231)

growth/size/body
• body weight is reduced from 14 days of age onward (J:163231)
• body weight is reduced from 14 days of age onward (J:163231)

behavior/neurological
• between 1 and 2 months of age, mutants are unable to right themselves (J:163231)
• between 1 and 2 months of age, mutants are unable to right themselves (J:163231)
• mutants fail to show proper escape extension by splaying their hindlimbs upon elevation (J:163231)
• mutants fail to show proper escape extension by splaying their hindlimbs upon elevation (J:163231)
• mutants exhibit body tremors by 21 days of age (J:163231)
• however, there is no evidence of muscle atrophy (J:163231)
• mutants exhibit body tremors by 21 days of age (J:163231)
• however, there is no evidence of muscle atrophy (J:163231)
• mutants develop an aberrant "swimming" gait (J:163231)
• by 21 days of age, mutants have difficulty walking faster than 10 cm/s and recruiting their hindlimbs (J:163231)
• mutants develop an aberrant "swimming" gait (J:163231)
• by 21 days of age, mutants have difficulty walking faster than 10 cm/s and recruiting their hindlimbs (J:163231)

nervous system
• brain weight is lower at 1 month of age (J:163231)
• brain weight is lower at 1 month of age (J:163231)
• in the brainstem, eosinphilic aggregates are found mostly within the pontine nuclei and midbrain tegmentum, including the red nucleus (J:163231)
• in the brainstem, eosinphilic aggregates are found mostly within the pontine nuclei and midbrain tegmentum, including the red nucleus (J:163231)
• eosinphilic aggregates (J:163231)
• eosinphilic aggregates (J:163231)
• reactive gliosis is seen in the anterior horn of the spinal cord (J:163231)
• reactive gliosis is seen in the anterior horn of the spinal cord (J:163231)
• reactive astrocytosis is seen in the gray matter of the spinal cord (J:163231)
• reactive astrocytosis is seen in the gray matter of the spinal cord (J:163231)
• mutants exhibit neuronal cytoplasmic eosinophilic aggregates in spinal motor neurons (J:163231)
• about 10% of neurons in the anterior horn of the spinal cord had eosinophilic aggregates, with fewer in the posterior horn and brainstem (J:163231)
• motoneuron eosinophilic aggregates are composed of abnormal mitochondrial clusters; mitochondria have features suggestive of degeneration, with decreased cristae and vacuoles within the mitochondrial matrix (J:163231)
• mutants exhibit neuronal cytoplasmic eosinophilic aggregates in spinal motor neurons (J:163231)
• about 10% of neurons in the anterior horn of the spinal cord had eosinophilic aggregates, with fewer in the posterior horn and brainstem (J:163231)
• motoneuron eosinophilic aggregates are composed of abnormal mitochondrial clusters; mitochondria have features suggestive of degeneration, with decreased cristae and vacuoles within the mitochondrial matrix (J:163231)
• degenerating neurites, axons and neurons are seen in the spinal cord (J:163231)
• degenerating neurites, axons and neurons are seen in the spinal cord (J:163231)
• degenerating mitochondria, and autophagic vacuoles, are seen within swollen axons in the spinal cord white matter (J:163231)
• degenerating mitochondria, and autophagic vacuoles, are seen within swollen axons in the spinal cord white matter (J:163231)
• in the spinal cord (J:163231)
• in the spinal cord (J:163231)
• vacuolization of myelin and myelin ovoids indicate myelin degeneration in anterolateral funiculi of the spinal cord (J:163231)
• vacuolization of myelin and myelin ovoids indicate myelin degeneration in anterolateral funiculi of the spinal cord (J:163231)


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
02/02/2016
MGI 6.02
The Jackson Laboratory