Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cybbtm1Din mutation
(1 available);
any
Cybb mutation
(42 available)
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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mortality/aging
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• life span improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur
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behavior/neurological
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• used as a measure of paralysis
• improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur
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• reached end stage paralysis later than mice only carrying Tg(SOD1*G93A)dl1Gur
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nervous system
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• improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur
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• 50% more anterior horn motor neurons in the anterior horn of the spinal cord than in mice only carrying Tg(SOD1*G93A)dl1Gur
• more myelinated axons in the 5th lumbar anterior roots than in mice only carrying Tg(SOD1*G93A)dl1Gur
• more innervated end plates than in mice only carrying Tg(SOD1*G93A)dl1Gur
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muscle
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• in the fibularis and peroneus longus
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mir206tm1Eno mutation
(0 available);
any
Mir206 mutation
(3 available)
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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mortality/aging
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• average death at 244 days compared to 266 days in mice carrying the transgene alone
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skeleton
muscle
behavior/neurological
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• accelerated compared to transgene alone
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nervous system
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• neuromuscular junctions are disorganized and show imperfect colocalization of nerve and postsynaptic sites
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mt3tm1Rpa mutation
(2 available);
any
Mt3 mutation
(5 available)
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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mortality/aging
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• markedly reduced survival, by about 20%
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behavior/neurological
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• severe declines in motor function starting around 5-6 months of age
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nervous system
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• becomes severe after mice begin to display behavioral symptoms
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Eno2-Hgf)1Tnak mutation
(0 available)
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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nervous system
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• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
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• decreased numbers of reactive astrocytes compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
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• less atrophic facial and hypoglossal motoneurons at 8 months of age
• the mean numbers of facial and hypoglossal motoneurons are larger than that of the Tg(SOD1*G93A)dl1Gur mice, and are almost the same as that of wild type mice
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immune system
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• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
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hematopoietic system
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• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
Tg(Thy1-SOD1*G93A)T3Hgrd mutation
(1 available)
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behavior/neurological
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• inability to perform hanging wire test
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muscle
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• first sign of disease is forelimb weakness
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• the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days
• animals show forelimb onset of disease
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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*G93A)dl1Gur mutation
(2 available)
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mortality/aging
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• mean survival age is 36 days
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growth/size/body
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• normal growth for the first five days after birth but weight is significantly reduced relative to controls at 6 days
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behavior/neurological
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• no neurological phenotype through 7 days of age but tremors appear around day 11
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• marked weakness and declining forelimb strength
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• shorter stride relative to controls at days 13-15
• maximum stride length at 20 days of age and becoming shorter thereafter
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muscle
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• spasticity with extensor hindlimb posturing appears around day 11
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nervous system
N |
• no vacuolation in dorsal root ganglia neurons
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• vacuoles found in spinal motor neurons at day seven and throughout the brainstem in certain nerves
• dilated vacuoles in lumbar spinal neurons by day 22
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Allelic Composition |
Tg(SOD1*G93A)dl1Gur/0
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Genetic Background |
involves: C57BL/6 * C57BL/6J * SJL/J |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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nervous system
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• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age
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• increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age
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• lower numbers of facial and hypoglossal motoneurons at 8 months of age
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• atrophic facial and hypoglossal motoneurons at 8 months of age
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immune system
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• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age
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hematopoietic system
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• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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mortality/aging
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• most symptomatic animals die at <300 days of age
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behavior/neurological
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• inability to perform hanging wire test
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muscle
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• first sign of disease is forelimb weakness
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• the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days
• animals show forelimb onset of disease
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cellular
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• vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals
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nervous system
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation
(2 available)
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mortality/aging
nervous system
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• mice develop neurofilament-rich Lewy-body like inclusions in the perikarya and proximal axons of the spinal cord motorneurons at day 180 that increase with age but are not as prominent as in Tg(SOD1*G93A)1Gur mice
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• at day 200, both fast and slow axonal transport are impaired in the ventral root compared to in wild-type axons
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behavior/neurological
muscle