Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(SOD1*)D-14Dbo mutation
(1 available)
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behavior/neurological
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• mice develop hindlimb paralysis between 12 and 20 months of age
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nervous system
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• within surviving motor neurons, wispy fibrillar ubiquitin, mutant SOD1, and Thioflavin S positive inclusions are seen that do not contain wild-type SOD1
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(SOD1*G85R)#Roos mutation
(0 available)
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mortality/aging
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• mean survival is 185 days
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behavior/neurological
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• progressive paralysis
• earlier onset (at about 131 days of age) of disease than single Tg(SOD1*G85R)#Roos mutants (at around 307 days of age), however disease duration is similar
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growth/size/body
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• weight loss begins at 131 days of age
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nervous system
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• in the anterior horn of the spinal cord at 150 days of age
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• SOD1-immunoreactive aggregates (of G85R mutant and wild-type SOD1 heterodimers, wild-type SOD1 homodimers and G85R mutant homodimers) are seen in motor neuron cells in the anterior horn at 150 days of age
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• loss of motor neurons at 150 days of age and loss of motor neuron connections with muscle in the lumbar spinal cord anterior horn
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(Thy1-SOD1*G93A)T3Hgrd mutation
(1 available)
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nervous system
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• ubiquinated SOD1 aggregates are seen at >45 weeks
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• muscle denervation is seen at end-stage of disease (<80 weeks)
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• motor neuron loss characterizes end-stage of disease (<80 weeks)
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• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks
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behavior/neurological
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• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs
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muscle
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• develops at about 1 year of age
• reach end-stage of disease before 80 weeks
• 60% of animals show hindlimb onset of disease while subset displays forelimb onset with short disease duration
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(SOD1*G93A)1Gur mutation
(4 available)
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mortality/aging
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• lifespan is on average 117.7 days
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nervous system
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• mutants exhibit exacerbated amyotrophic lateral sclerosis-like phenotypes compared to single Tg(SOD1*G93A)1Gur mice, with onset of disease on average at 76.8 days of age
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(SOD1*A4V)A1073Gur mutation
(0 available)
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mortality/aging
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• lifespan is on average 318.5 days
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nervous system
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• mutants exhibit protein aggregates in spinal cords
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• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
Tg(SOD1*L126Z)#Deng mutation
(0 available)
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mortality/aging
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• lifespan is on average 201.4 days
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nervous system
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• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords
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• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days
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cellular
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• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-CCS)17Jlel mutation
(1 available)
Tg(SOD1)2Gur mutation
(2 available)
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behavior/neurological
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• no neurological disease is observed
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Allelic Composition |
Tg(SOD1)2Gur/0
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Genetic Background |
involves: C57BL/6 * CBA * SJL |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
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nervous system
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• ubiquinated SOD1 aggregates accumulate in oligodendrocytes of spinal cords of aged mice (>70 weeks) but a much lower density than in double transgenic T3/SOD1 animals
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Allelic Composition |
Tg(SOD1)2Gur/0
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Genetic Background |
involves: C57BL/6 * SJL |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation
(2 available)
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Amyotrophic lateral sclerosis-like lesions in the spinal cord of Tg(SOD1)2Gur/0 and Tg(SOD1-G93A)1Gur/0 mice
nervous system
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• mild swelling of axons traveling toward the anterior roots at 232 days of age
(J:78629)
• numerous small vacuoles in the axoplasm of some swollen axons
(J:78629)
• never show any clinical signs of disease at up to 300 days of age
(J:78630)
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• develop neurofilament-rich spheroids in the spinal cords at much later time (132 days of age) points than Tg(SOD1-G93A)1Gur mutants, however do not appear to develop motor neuron disease
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