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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-TARDBP*A315T)95Balo
transgene insertion 95, Robert Baloh
MGI:4358722
Summary 8 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-TARDBP*A315T)95Balo/0 (129S1/SvImJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5526998
tg2
Tg(Prnp-TARDBP*A315T)95Balo/0 (ALR/LtJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5526997
tg3
Tg(Prnp-TARDBP*A315T)95Balo/0 (C3H/HeJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5527001
tg4
Tg(Prnp-TARDBP*A315T)95Balo/0 (FVB/NJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5526996
tg5
Tg(Prnp-TARDBP*A315T)95Balo/0 (PWK/PhJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5526999
tg6
Tg(Prnp-TARDBP*A315T)95Balo/0 (WSB/EiJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1 MGI:5527000
tg7
Tg(Prnp-TARDBP*A315T)95Balo/0 B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J MGI:5527002
tg8
Tg(Prnp-TARDBP*A315T)95Balo/0 involves: C57BL/6 * CBA MGI:4361717


Genotype
MGI:5526998
tg1
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(129S1/SvImJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5526997
tg2
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(ALR/LtJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5527001
tg3
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(C3H/HeJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5526996
tg4
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(FVB/NJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by 50-90 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5526999
tg5
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(PWK/PhJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5527000
tg6
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
(WSB/EiJ x B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)
• Background Sensitivity: survival in F1 males is increased by more than 340 days as compared to males on the C57BL/6J background (J:203041)

digestive/alimentary system
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)
• all F1 mice die with visible signs of gut pathology similar to mice on the C57BL/6J background (J:203041)




Genotype
MGI:5527002
tg7
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• late onset difficulty in righting occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset difficulty in righting occurs suddenly and progresses rapidly especially in males (J:203041)
• mild deficit in the hindlimb reflex is observed in both male and female mice (J:203041)
• mild deficit in the hindlimb reflex is observed in both male and female mice (J:203041)
• time-to-fall as measured by the hanging wire test is significantly decreased in transgenic males as compared to wild type littermates (J:203041)
• time-to-fall as measured by the hanging wire test is significantly decreased in transgenic males as compared to wild type littermates (J:203041)
• late onset immobility occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset immobility occurs suddenly and progresses rapidly especially in males (J:203041)
• both male and female mice exhibit a tendency to drag their tails when walking (J:203041)
• both male and female mice exhibit a tendency to drag their tails when walking (J:203041)

digestive/alimentary system
• progressive decrease in number of fecal pellets excreted (J:203041)
• progressive decrease in number of fecal pellets excreted (J:203041)
• gut motility is significantly decreased beginning at day 60 in males and progresses to 282% of controls by day 90 (J:203041)
• gut motility is significantly decreased in females but at later age than males and with less severity (J:203041)
• gut motility is significantly decreased beginning at day 60 in males and progresses to 282% of controls by day 90 (J:203041)
• gut motility is significantly decreased in females but at later age than males and with less severity (J:203041)
• lower GI tract develops swelling, intra-intestinal coagulated blood, and necrotic tissue, however, pathology of upper digestive tract is normal (J:203041)
• lower GI tract develops swelling, intra-intestinal coagulated blood, and necrotic tissue, however, pathology of upper digestive tract is normal (J:203041)

growth/size/body
• weight loss becomes significant at post-natal day 85 in males and post-natal day 127 in females (J:203041)
• weight loss becomes significant at post-natal day 85 in males and post-natal day 127 in females (J:203041)
• late onset swollen and tender abdomen occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset swollen and tender abdomen occurs suddenly and progresses rapidly especially in males (J:203041)

homeostasis/metabolism
• late onset dehydration occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset dehydration occurs suddenly and progresses rapidly especially in males (J:203041)

integument
• late onset unkempt haircoat occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset unkempt haircoat occurs suddenly and progresses rapidly especially in males (J:203041)

mortality/aging
• ovariectomy decreases female median survival by 94 days (J:203041)
• male castration decreases median survival by 10 days (J:203041)
• ovariectomy decreases female median survival by 94 days (J:203041)
• male castration decreases median survival by 10 days (J:203041)
• Background Sensitivity: death occurs at a median of 108 days in males (J:203041)
• Background Sensitivity: death occurs at a median of 185 days in females (J:203041)
• Background Sensitivity: death occurs at a median of 108 days in males (J:203041)
• Background Sensitivity: death occurs at a median of 185 days in females (J:203041)

nervous system
• ganglions in the superior mesentery appear smaller than control ganglions (J:203041)
• ganglions exhibit an increased percentage of condensed nuclei (J:203041)
• ganglions in the superior mesentery appear smaller than control ganglions (J:203041)
• ganglions exhibit an increased percentage of condensed nuclei (J:203041)
• decreased AChE staining in the ganglion and intermodal strands of the myenteric plexus of the colon (J:203041)
• decreased AChE staining in the ganglion and intermodal strands of the myenteric plexus of the colon (J:203041)
• 80% decrease in the number of neurons per ganglion in the myenteric plexus of the colon, but not the duodenum, of 90-150 day old mice (J:203041)
• 80% decrease in the number of neurons per ganglion in the myenteric plexus of the colon, but not the duodenum, of 90-150 day old mice (J:203041)
• reactive astrocytosis observed in spinal cord lumbar sections from 80 day old males (J:203041)
• reactive astrocytosis observed in spinal cord lumbar sections from 80 day old males (J:203041)
• the large motor neurons of some males exhibit abnormally shaped nuclei (J:203041)
• the large motor neurons of some males exhibit abnormally shaped nuclei (J:203041)
(J:203041)
(J:203041)
• small, but significant decrease in axon number and diameter in the sensory branch of the femoral nerve in 3 and 5 month old males (J:203041)
• small, but significant decrease in axon number and diameter in the sensory branch of the femoral nerve in 3 and 5 month old males (J:203041)
• some motor neurons have ubiquitin-positive cytoplasmic inclusions (J:203041)
• some motor neurons have ubiquitin-positive cytoplasmic inclusions (J:203041)

skeleton
• late onset kyphosis occurs suddenly and progresses rapidly especially in males (J:203041)
• late onset kyphosis occurs suddenly and progresses rapidly especially in males (J:203041)




Genotype
MGI:4361717
tg8
Allelic
Composition
Tg(Prnp-TARDBP*A315T)95Balo/0
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)95Balo mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• average survival is 154 days; at end-stage, mice die spontaneously or are euthanized when they lose righting reflex or can no longer obtain food and water (J:153197)
• average survival is 154 days; at end-stage, mice die spontaneously or are euthanized when they lose righting reflex or can no longer obtain food and water (J:153197)

growth/size/body
• mice begin losing weight around 4.5 months of age (J:153197)
• mice begin losing weight around 4.5 months of age (J:153197)

behavior/neurological
• at 3-4 months mice develop abnormal gait; around 4.5 months, mice exhibit a "swimming" gait when they lose ability to support their body weight but still use their limbs for propulsion to slide on their stomachs (J:153197)
• at 3-4 months mice develop abnormal gait; around 4.5 months, mice exhibit a "swimming" gait when they lose ability to support their body weight but still use their limbs for propulsion to slide on their stomachs (J:153197)

nervous system
• activation of astrocytes is detected in layer 5 with reactive astrocytosis seen around degenerating neurons (J:153197)
• activation of astrocytes is detected in layer 5 with reactive astrocytosis seen around degenerating neurons (J:153197)
• late-stage mice have cytoplasmic accumulation of ubiquinated proteins in neurons of cortical layer 5; these neurons are prominent in the motor cortex and are also present in orbital, cingulated, sensory and other cortical regions (J:153197)
• no ubiquinated protein aggregates are observed in the caudate/putamen, substantia nigra, thalamus or other structures at any stage (J:153197)
• late-stage mice have cytoplasmic accumulation of ubiquinated proteins in neurons of cortical layer 5; these neurons are prominent in the motor cortex and are also present in orbital, cingulated, sensory and other cortical regions (J:153197)
• no ubiquinated protein aggregates are observed in the caudate/putamen, substantia nigra, thalamus or other structures at any stage (J:153197)
• neuron loss in cortical layer 5 (J:153197)
• neuron loss in cortical layer 5 (J:153197)
• fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns (J:153197)
• femoral motor and sensory nerves shows loss of axons with ongoing axonal degeneration in the motor branch (J:153197)
• fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns (J:153197)
• femoral motor and sensory nerves shows loss of axons with ongoing axonal degeneration in the motor branch (J:153197)
• fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns (J:153197)
• fewer axons are observed in the lower thoracic spinal cord with numerous degenerating axons being seen in dorsal corticospinal tract and lateral columns (J:153197)
• in end-stage mice, about a 20% loss of spinal motor neurons is observed (J:153197)
• in end-stage mice, about a 20% loss of spinal motor neurons is observed (J:153197)
• presence of ubiquitinated protein accumulations is detected preferentially in large neurons of the dorsal horn as well as scattered interneurons (J:153197)
• presence of ubiquitinated protein accumulations is detected preferentially in large neurons of the dorsal horn as well as scattered interneurons (J:153197)

muscle
• end-stage mice have scattered and grouped atrophic muscle fibers, characteristic of muscle denervation (J:153197)
• end-stage mice have scattered and grouped atrophic muscle fibers, characteristic of muscle denervation (J:153197)
• atrophic muscle fibers are observed in end-stage mice (J:153197)
• atrophic muscle fibers are observed in end-stage mice (J:153197)
• electromyography in end-stage mice shows numerous fibrillation potentials indicative of loss of muscle fiber innervation and fasciculations, which are spontaneous firing of motor units often seen with human motor neuron diseases; in presymptomatic and early-stage mice, electromyography is normal (J:153197)
• electromyography in end-stage mice shows numerous fibrillation potentials indicative of loss of muscle fiber innervation and fasciculations, which are spontaneous firing of motor units often seen with human motor neuron diseases; in presymptomatic and early-stage mice, electromyography is normal (J:153197)
• around 4.5 months, mice can no longer support their body weight (J:153197)
• around 4.5 months, mice can no longer support their body weight (J:153197)





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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory