Phenotypes associated with this allele

• Allele Symbol: Tg(SOD1)2Gur
• Allele Name: transgene insertion 2, Mark E Gurney
• Allele ID: 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

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

behavior/neurological

hindlimb paralysis ( J:218091 )

• mice develop hindlimb paralysis between 12 and 20 months of age

nervous system

abnormal motor neuron morphology ( 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

motor neuron degeneration ( J:218091 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM: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

mortality/aging

premature death ( J:147156 )

• mean survival is 185 days

behavior/neurological

hindlimb paralysis ( J:147156 )

• 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 ( J:147156 )

• weight loss begins at 131 days of age

nervous system

astrocytosis ( J:147156 )

• in the anterior horn of the spinal cord at 150 days of age

abnormal motor neuron morphology ( 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

motor neuron degeneration ( 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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM: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

nervous system

abnormal oligodendrocyte morphology ( J:134095 )

• ubiquinated SOD1 aggregates are seen at >45 weeks

abnormal innervation pattern to muscle ( J:134095 )

• muscle denervation is seen at end-stage of disease (<80 weeks)

decreased motor neuron number ( J:134095 )

• motor neuron loss characterizes end-stage of disease (<80 weeks)

abnormal dendrite morphology ( J:134095 )

• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks

behavior/neurological

paralysis ( J:134095 )

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

muscle

muscle weakness ( J:134095 )

• 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

mortality/aging

premature death ( J:109458 )

• lifespan is on average 117.7 days

nervous system

abnormal nervous system physiology ( 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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109458

Genotype
MGI:5449900

cx5

• Allelic Composition: Tg(SOD1)2Gur/0; Tg(SOD1*A4V)A1073Gur/0
• Genetic Background: involves: C57BL/6 * SJL

mortality/aging

premature death ( J:109458 )

• lifespan is on average 318.5 days

nervous system

abnormal spinal cord grey matter morphology ( J:109458 )

• mutants exhibit protein aggregates in spinal cords

abnormal nervous system physiology ( J:109458 )

• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109458

Genotype
MGI:5449903

cx6

• Allelic Composition: Tg(SOD1)2Gur/0; Tg(SOD1*L126Z)#Deng/0
• Genetic Background: involves: C57BL/6 * SJL

mortality/aging

premature death ( J:109458 )

• lifespan is on average 201.4 days

nervous system

abnormal spinal cord grey matter morphology ( J:109458 )

• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords

abnormal nervous system physiology ( J:109458 )

• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days

cellular

abnormal mitochondrial crista morphology ( J:109458 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109458

Genotype
MGI:5493309

cx7

• Allelic Composition: Tg(Prnp-CCS)17Jlel/?; Tg(SOD1)2Gur/?
• Genetic Background: involves: C57BL/6 * SJL

behavior/neurological

behavior/neurological phenotype ( J:120361 )

N • no neurological disease is observed

Genotype
MGI:3785394

tg8

• Allelic Composition: Tg(SOD1)2Gur/0
• Genetic Background: involves: C57BL/6 * CBA * SJL

nervous system

abnormal oligodendrocyte morphology ( 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

Genotype
MGI:3688105

tg9

• Allelic Composition: Tg(SOD1)2Gur/0
• Genetic Background: involves: C57BL/6 * SJL

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

nervous system

abnormal axon morphology ( J:78629 , J:78630 )

• 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)

abnormal spinal cord morphology ( 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