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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 (J:218091)
• mice develop hindlimb paralysis between 12 and 20 months of age (J:218091)

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 (J:218091)
• within surviving motor neurons, wispy fibrillar ubiquitin, mutant SOD1, and Thioflavin S positive inclusions are seen that do not contain wild-type SOD1 (J:218091)

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 (J:147156)
• mean survival is 185 days (J:147156)

behavior/neurological
• progressive paralysis (J:147156)
• 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 (J:147156)
• progressive paralysis (J:147156)
• 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 (J:147156)

growth/size/body
• weight loss begins at 131 days of age (J:147156)
• weight loss begins at 131 days of age (J:147156)

nervous system
• in the anterior horn of the spinal cord at 150 days of age (J:147156)
• in the anterior horn of the spinal cord at 150 days of age (J:147156)
• 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 (J:147156)
• 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 (J:147156)
• loss of motor neurons at 150 days of age and loss of motor neuron connections with muscle in the lumbar spinal cord anterior horn (J:147156)
• loss of motor neurons at 150 days of age and loss of motor neuron connections with muscle in the lumbar spinal cord anterior horn (J:147156)

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 (J:134095)
• ubiquinated SOD1 aggregates are seen at >45 weeks (J:134095)
• muscle denervation is seen at end-stage of disease (<80 weeks) (J:134095)
• muscle denervation is seen at end-stage of disease (<80 weeks) (J:134095)
• motor neuron loss characterizes end-stage of disease (<80 weeks) (J:134095)
• motor neuron loss characterizes end-stage of disease (<80 weeks) (J:134095)
• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks (J:134095)
• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks (J:134095)

behavior/neurological
• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs (J:134095)
• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs (J:134095)

muscle
• develops at about 1 year of age (J:134095)
• reach end-stage of disease before 80 weeks (J:134095)
• 60% of animals show hindlimb onset of disease while subset displays forelimb onset with short disease duration (J:134095)
• develops at about 1 year of age (J:134095)
• reach end-stage of disease before 80 weeks (J:134095)
• 60% of animals show hindlimb onset of disease while subset displays forelimb onset with short disease duration (J:134095)




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 (J:109458)
• lifespan is on average 117.7 days (J:109458)

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 (J:109458)
• 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 (J:109458)

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 (J:109458)
• lifespan is on average 318.5 days (J:109458)

nervous system
• mutants exhibit protein aggregates in spinal cords (J:109458)
• mutants exhibit protein aggregates in spinal cords (J:109458)
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days (J:109458)
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days (J:109458)

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 (J:109458)
• lifespan is on average 201.4 days (J:109458)

nervous system
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords (J:109458)
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords (J:109458)
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days (J:109458)
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days (J:109458)

cellular
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae (J:109458)
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae (J:109458)

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 (J:120361)
• no neurological disease is observed (J:120361)




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 (J:134095)
• 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 (J:134095)




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)
• 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)
• 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 (J:76718)
• 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 (J:76718)





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