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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1)2Gur
transgene insertion 2, Mark E Gurney
MGI:2448770
Summary 9 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(SOD1)2Gur/0
Tg(SOD1*)D-14Dbo/0
involves: C3H/HeJ * C57BL/6 * C57BL/6J * SJL MGI:5621175
cx2
Tg(SOD1)2Gur/0
Tg(SOD1*G85R)#Roos/0
involves: C57BL/6 * C57BL/6J * SJL MGI:5448549
cx3
Tg(SOD1)2Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0
involves: C57BL/6 * CBA * FVB * SJL MGI:3785393
cx4
Tg(SOD1)2Gur/0
Tg(SOD1*G93A)1Gur/0
involves: C57BL/6 * SJL MGI:5449898
cx5
Tg(SOD1)2Gur/0
Tg(SOD1*A4V)A1073Gur/0
involves: C57BL/6 * SJL MGI:5449900
cx6
Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0
involves: C57BL/6 * SJL MGI:5449903
cx7
Tg(Prnp-CCS)17Jlel/?
Tg(SOD1)2Gur/?
involves: C57BL/6 * SJL MGI:5493309
tg8
Tg(SOD1)2Gur/0 involves: C57BL/6 * CBA * SJL MGI:3785394
tg9
Tg(SOD1)2Gur/0 involves: C57BL/6 * SJL MGI:3688105


Genotype
MGI:5621175
cx1
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*)D-14Dbo/0
Genetic
Background
involves: C3H/HeJ * C57BL/6 * C57BL/6J * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice develop hindlimb paralysis between 12 and 20 months of age

nervous system
• within surviving motor neurons, wispy fibrillar ubiquitin, mutant SOD1, and Thioflavin S positive inclusions are seen that do not contain wild-type SOD1

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:218091




Genotype
MGI:5448549
cx2
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*G85R)#Roos/0
Genetic
Background
involves: C57BL/6 * C57BL/6J * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean survival is 185 days

behavior/neurological
• 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

growth/size/body
• weight loss begins at 131 days of age

nervous system
• in the anterior horn of the spinal cord at 150 days of age
• 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
• loss of motor neurons at 150 days of age and loss of motor neuron connections with muscle in the lumbar spinal cord anterior horn

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:147156




Genotype
MGI:3785393
cx3
Allelic
Composition
Tg(SOD1)2Gur/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)2Gur mutation (2 available)
Tg(Thy1-SOD1*G93A)T3Hgrd mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• ubiquinated SOD1 aggregates are seen at >45 weeks
• muscle denervation is seen at end-stage of disease (<80 weeks)
• motor neuron loss characterizes end-stage of disease (<80 weeks)
• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks

behavior/neurological
• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs

muscle
• 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




Genotype
MGI:5449898
cx4
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*G93A)1Gur/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation (2 available)
Tg(SOD1*G93A)1Gur mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan is on average 117.7 days

nervous system
• 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

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458




Genotype
MGI:5449900
cx5
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*A4V)A1073Gur/0
Genetic
Background
involves: C57BL/6 * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan is on average 318.5 days

nervous system
• mutants exhibit protein aggregates in spinal cords
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458




Genotype
MGI:5449903
cx6
Allelic
Composition
Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0
Genetic
Background
involves: C57BL/6 * SJL
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lifespan is on average 201.4 days

nervous system
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days

cellular
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458




Genotype
MGI:5493309
cx7
Allelic
Composition
Tg(Prnp-CCS)17Jlel/?
Tg(SOD1)2Gur/?
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)2Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
N
• no neurological disease is observed




Genotype
MGI:3785394
tg8
Allelic
Composition
Tg(SOD1)2Gur/0
Genetic
Background
involves: C57BL/6 * CBA * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• 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




Genotype
MGI:3688105
tg9
Allelic
Composition
Tg(SOD1)2Gur/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1)2Gur mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Amyotrophic lateral sclerosis-like lesions in the spinal cord of Tg(SOD1)2Gur/0 and Tg(SOD1-G93A)1Gur/0 mice

nervous system
• 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)
• 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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last database update
09/27/2016
MGI 6.05
The Jackson Laboratory