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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1*G93A)dl1Gur
transgene insertion dl 1, Mark E Gurney
MGI:2671112
Summary 9 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Cybbtm1Din/Y
Tg(SOD1*G93A)dl1Gur/0
B6.Cg-Cybbtm1Din Tg(SOD1*G93A)dl1Gur MGI:4440461
cx2
Mir206tm1Eno/Mir206tm1Eno
Tg(SOD1*G93A)dl1Gur/0
involves: 129S/SvEv * C57BL/6 * SJL MGI:4418180
cx3
Mt3tm1Rpa/Mt3tm1Rpa
Tg(SOD1*G93A)dl1Gur/0
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:3574995
cx4
Tg(Eno2-Hgf)1Tnak/0
Tg(SOD1*G93A)dl1Gur/0
involves: C57BL/6 * C57BL/6J * SJL/J MGI:4835971
cx5
Tg(SOD1*G93A)dl1Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0
involves: C57BL/6 * CBA * FVB * SJL MGI:3785391
cx6
Tg(Prnp-CCS)17Jlel/?
Tg(SOD1*G93A)dl1Gur/?
involves: C57BL/6 * SJL MGI:5493308
tg7
Tg(SOD1*G93A)dl1Gur/0 involves: C57BL/6 * C57BL/6J * SJL/J MGI:4835972
tg8
Tg(SOD1*G93A)dl1Gur/0 involves: C57BL/6 * CBA * SJL MGI:3785392
tg9
Tg(SOD1*G93A)dl1Gur/0 involves: C57BL/6 * SJL MGI:3813319


Genotype
MGI:4440461
cx1
Allelic
Composition
Cybbtm1Din/Y
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
B6.Cg-Cybbtm1Din Tg(SOD1*G93A)dl1Gur
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cybbtm1Din mutation (1 available); any Cybb mutation (9 available)
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• life span improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur

behavior/neurological
• used as a measure of paralysis
• improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur
• reached end stage paralysis later than mice only carrying Tg(SOD1*G93A)dl1Gur

nervous system
• improved relative to mice only carrying Tg(SOD1*G93A)dl1Gur
• 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

muscle
• in the fibularis and peroneus longus

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:111782




Genotype
MGI:4418180
cx2
Allelic
Composition
Mir206tm1Eno/Mir206tm1Eno
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: 129S/SvEv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mir206tm1Eno mutation (0 available); any Mir206 mutation (0 available)
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• average death at 244 days compared to 266 days in mice carrying the transgene alone

skeleton

muscle
• at 7.5 months of age

behavior/neurological
• accelerated compared to transgene alone

nervous system
• neuromuscular junctions are disorganized and show imperfect colocalization of nerve and postsynaptic sites




Genotype
MGI:3574995
cx3
Allelic
Composition
Mt3tm1Rpa/Mt3tm1Rpa
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mt3tm1Rpa mutation (1 available); any Mt3 mutation (1 available)
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• markedly reduced survival, by about 20%

behavior/neurological
• severe declines in motor function starting around 5-6 months of age

nervous system
• becomes severe after mice begin to display behavioral symptoms




Genotype
MGI:4835971
cx4
Allelic
Composition
Tg(Eno2-Hgf)1Tnak/0
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: C57BL/6 * C57BL/6J * SJL/J
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
• decreased numbers of reactive astrocytes compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age
• 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

immune system
• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

hematopoietic system
• decreased numbers of microglia compared with Tg(SOD1*G93A)dl1Gur mice in facial and hypoglossal nuclei at 8 months of age




Genotype
MGI:3785391
cx5
Allelic
Composition
Tg(SOD1*G93A)dl1Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0
Genetic
Background
involves: C57BL/6 * CBA * FVB * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• inability to perform hanging wire test

muscle
• first sign of disease is forelimb weakness
• 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




Genotype
MGI:5493308
cx6
Allelic
Composition
Tg(Prnp-CCS)17Jlel/?
Tg(SOD1*G93A)dl1Gur/?
Genetic
Background
involves: C57BL/6 * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean survival age is 36 days

growth/size/body
• normal growth for the first five days after birth but weight is significantly reduced relative to controls at 6 days

behavior/neurological
• no neurological phenotype through 7 days of age but tremors appear around day 11
• around day 11
• marked weakness and declining forelimb strength
• shorter stride relative to controls at days 13-15
• maximum stride length at 20 days of age and becoming shorter thereafter

muscle
• spasticity with extensor hindlimb posturing appears around day 11

nervous system
N
• no vacuolation in dorsal root ganglia neurons
• 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




Genotype
MGI:4835972
tg7
Allelic
Composition
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: C57BL/6 * C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age
• increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age
• lower numbers of facial and hypoglossal motoneurons at 8 months of age
• atrophic facial and hypoglossal motoneurons at 8 months of age

immune system
• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

hematopoietic system
• increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age




Genotype
MGI:3785392
tg8
Allelic
Composition
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: C57BL/6 * CBA * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most symptomatic animals die at <300 days of age

behavior/neurological
• inability to perform hanging wire test

muscle
• first sign of disease is forelimb weakness
• 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

cellular
• vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals

nervous system




Genotype
MGI:3813319
tg9
Allelic
Composition
Tg(SOD1*G93A)dl1Gur/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G93A)dl1Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean 266 day

nervous system
• 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
• at day 200, both fast and slow axonal transport are impaired in the ventral root compared to in wild-type axons

behavior/neurological
• develops at day 200
• at day 250 (J:106420)

muscle
• develops at day 200

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:106420





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last database update
04/19/2016
MGI 6.03
The Jackson Laboratory