Phenotypes associated with this allele

• Allele Symbol: Tg(SOD1*G93A)^dl1Gur
• Allele Name: transgene insertion dl 1, Mark E Gurney
• Allele ID: MGI:2671112
• Summary: 10 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Cybb^tm1Din/Y Tg(SOD1*G93A)^dl1Gur/0	B6.Cg-Cybb^tm1Din Tg(SOD1*G93A)^dl1Gur	MGI:4440461
cx2	Mt3^tm1Rpa/Mt3^tm1Rpa Tg(SOD1*G93A)^dl1Gur/0	involves: 129S7/SvEvBrd * C57BL/6 * SJL	MGI:3574995
cx3	Mir206^tm1Eno/Mir206^tm1Eno Tg(SOD1*G93A)^dl1Gur/0	involves: 129S/SvEv * C57BL/6 * SJL	MGI:4418180
cx4	Tardbp^m1H/Tardbp^+ Tg(SOD1*G93A)^dl1Gur/0	involves: C3H/HeH * C57BL/6 * C57BL/6J * SJL	MGI:5575774
cx5	Tg(Eno2-Hgf)1Tnak/0 Tg(SOD1*G93A)^dl1Gur/0	involves: C57BL/6 * C57BL/6J * SJL/J	MGI:4835971
cx6	Tg(SOD1*G93A)^dl1Gur/0 Tg(Thy1-SOD1*G93A)T3Hgrd/0	involves: C57BL/6 * CBA * FVB * SJL	MGI:3785391
cx7	Tg(Prnp-CCS)17Jlel/? Tg(SOD1*G93A)^dl1Gur/?	involves: C57BL/6 * SJL	MGI:5493308
tg8	Tg(SOD1*G93A)^dl1Gur/0	involves: C57BL/6 * C57BL/6J * SJL/J	MGI:4835972
tg9	Tg(SOD1*G93A)^dl1Gur/0	involves: C57BL/6 * CBA * SJL	MGI:3785392
tg10	Tg(SOD1*G93A)^dl1Gur/0	involves: C57BL/6 * SJL	MGI:3813319

Genotype
MGI:4440461

cx1

• Allelic Composition: Cybb^tm1Din/Y; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: B6.Cg-Cybb^tm1Din Tg(SOD1*G93A)^dl1Gur

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

mortality/aging

premature death ( J:111782 )

♂ • life span improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

behavior/neurological

abnormal righting response ( J:111782 )

♂ • used as a measure of paralysis

♂ • improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

paralysis ( J:111782 )

♂ • reached end stage paralysis later than mice only carrying Tg(SOD1*G93A)^dl1Gur

nervous system

neurodegeneration ( J:111782 )

♂ • improved relative to mice only carrying Tg(SOD1*G93A)^dl1Gur

axon degeneration ( J:111782 )

♂ • 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

increased skeletal muscle fiber size ( J:111782 )

♂ • in the fibularis and peroneus longus

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:111782

Genotype
MGI:3574995

cx2

• Allelic Composition: Mt3^tm1Rpa/Mt3^tm1Rpa; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6 * SJL

mortality/aging

premature death ( J:79760 )

• markedly reduced survival, by about 20%

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:79760 )

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

decreased grip strength ( J:79760 )

• reduced

nervous system

decreased motor neuron number ( J:79760 )

• becomes severe after mice begin to display behavioral symptoms

Genotype
MGI:4418180

cx3

• Allelic Composition: Mir206^tm1Eno/Mir206^tm1Eno; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * SJL

mortality/aging

premature death ( J:155806 )

• average death at 244 days compared to 266 days in mice carrying the transgene alone

skeleton

kyphosis ( J:155806 )

muscle

skeletal muscle atrophy ( J:155806 )

• at 7.5 months of age

behavior/neurological

hindlimb paralysis ( J:155806 )

• accelerated compared to transgene alone

nervous system

abnormal neuromuscular synapse morphology ( J:155806 )

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

Genotype
MGI:5575774

cx4

• Allelic Composition: Tardbp^m1H/Tardbp⁺; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C3H/HeH * C57BL/6 * C57BL/6J * SJL

mortality/aging

premature death ( J:211620 )

• as in Tg(SOD1*G93A)1Gur mice

muscle

decreased extensor digitorum longus weight ( J:211620 )

• compared with Tg(SOD1*G93A)1Gur mice

impaired muscle relaxation ( J:211620 )

• in the tibialis anterior compared with Tg(SOD1*G93A)1Gur mice

• however, relaxation time in the extensor digitorum longus is normal

abnormal muscle tone ( J:211620 )

• soft abdominal body tone as in Tardbp^m1H heterozygotes

behavior/neurological

decreased grip strength ( J:211620 )

• as in Tg(SOD1*G93A)1Gur mice

growth/size/body

decreased body weight ( J:211620 )

• as in Tg(SOD1*G93A)1Gur mice

nervous system

nervous system phenotype ( J:211620 )

N • mice exhibit normal numbers of motor neurons in the sciatic motor pool

limbs/digits/tail

decreased extensor digitorum longus weight ( J:211620 )

• compared with Tg(SOD1*G93A)1Gur mice

Genotype
MGI:4835971

cx5

• Allelic Composition: Tg(Eno2-Hgf)1Tnak/0; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * C57BL/6J * SJL/J

nervous system

microgliosis ( J:128738 )

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

astrocytosis ( J:128738 )

• decreased numbers of reactive astrocytes compared with Tg(SOD1*G93A)^dl1Gur mice in facial and hypoglossal nuclei at 8 months of age

abnormal motor neuron morphology ( J:128738 )

• 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

microgliosis ( J:128738 )

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

hematopoietic system

microgliosis ( J:128738 )

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

Genotype
MGI:3785391

cx6

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0; Tg(Thy1-SOD1*G93A)T3Hgrd/0
• Genetic Background: involves: C57BL/6 * CBA * FVB * SJL

behavior/neurological

impaired coordination ( J:134095 )

• inability to perform hanging wire test

muscle

muscle weakness ( J:134095 )

• first sign of disease is forelimb weakness

progressive muscle weakness ( J:134095 )

• 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

cx7

• Allelic Composition: Tg(Prnp-CCS)17Jlel/?; Tg(SOD1*G93A)^dl1Gur/?
• Genetic Background: involves: C57BL/6 * SJL

mortality/aging

premature death ( J:120361 )

• mean survival age is 36 days

growth/size/body

abnormal postnatal growth ( J:120361 )

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

behavior/neurological

tremors ( J:120361 )

• no neurological phenotype through 7 days of age but tremors appear around day 11

ataxia ( J:120361 )

• around day 11

decreased grip strength ( J:120361 )

• marked weakness and declining forelimb strength

short stride length ( J:120361 )

• shorter stride relative to controls at days 13-15

• maximum stride length at 20 days of age and becoming shorter thereafter

muscle

muscle hypertonia ( J:120361 )

• spasticity with extensor hindlimb posturing appears around day 11

nervous system

nervous system phenotype ( J:120361 )

N • no vacuolation in dorsal root ganglia neurons

abnormal hypoglossal nucleus morphology ( J:120361 )

• neuron vacuolation

abnormal motor neuron morphology ( J:120361 )

• 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

tg8

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * C57BL/6J * SJL/J

nervous system

microgliosis ( J:128738 )

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

astrocytosis ( J:128738 )

• increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age

decreased motor neuron number ( J:128738 )

• lower numbers of facial and hypoglossal motoneurons at 8 months of age

motor neuron degeneration ( J:128738 )

• atrophic facial and hypoglossal motoneurons at 8 months of age

immune system

microgliosis ( J:128738 )

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

hematopoietic system

microgliosis ( J:128738 )

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

Genotype
MGI:3785392

tg9

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * CBA * SJL

mortality/aging

premature death ( J:134095 )

• most symptomatic animals die at <300 days of age

behavior/neurological

impaired coordination ( J:134095 )

• inability to perform hanging wire test

muscle

muscle weakness ( J:134095 )

• first sign of disease is forelimb weakness

progressive muscle weakness ( J:134095 )

• 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

abnormal mitochondrial morphology ( J:134095 )

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

nervous system

astrocytosis ( J:134095 )

Genotype
MGI:3813319

tg10

• Allelic Composition: Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: C57BL/6 * SJL

mortality/aging

premature death ( J:155806 )

• mean 266 day

nervous system

abnormal neuron morphology ( J:106420 )

• 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

abnormal axonal transport ( J:106420 )

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

behavior/neurological

limb grasping ( J:106420 )

• develops at day 200

paralysis ( J:106420 , J:155806 )

• at day 250 (J:106420)

hindlimb paralysis ( J:155806 )

muscle

muscle weakness ( J:106420 )

• develops at day 200

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:106420