Mouse Genome Informatics
cx1
    Tg(Thy1-SOD1*G93A)T1Hgrd/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0

involves: C57BL/6 * CBA * FVB
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
growth/size
• at end-stage, most mice display severe weight loss (>30%)

behavior/neurological
• at end-stage, severe locomotor deficits are exhibited
• at end-stage, loss of ability to hang in hanging wire test
• loss of grip strength at end-stage of disease

nervous system
• accumulation of argyrophilic neuronal debris is seen throughout medullary, pontine, and mesencephalic reticular formation up to zona incerta
• signs of reactive gliosis are seen at disease end-stage
• animals not displaying motor abnormalities show ubiquinated neurites in spinal cord
• in mice at end-stage, denervation is observed at neuromuscular synapses
• appears restricted to brainstem and spinal cord
• accumulation of argyrophilic neuronal debris is seen in spinal cord of mice at end-stage of disease; similar amounts are seen between left and right, and lumbar and cervical segments
• loss of motor neurons is detected at disease end-stage

muscle
• exhibited by most mice (67%) before 2 years of age; onset is observed at 539 days to more than 730 days with end stage reached at 591 to greater than 730 days

cellular
N
• mice do not develop mitochondrial swelling and vacuolization like G1 or G1del mutants (J:134095)

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:134095


Mouse Genome Informatics
cx2
    Tg(SOD1*G93A)dl1Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0

involves: C57BL/6 * CBA * FVB * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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


Mouse Genome Informatics
cx3
    Tg(SOD1)2Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0

involves: C57BL/6 * CBA * FVB * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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


Mouse Genome Informatics
tg4
    Tg(Thy1-SOD1*G93A)T3Hgrd/Tg(Thy1-SOD1*G93A)T3Hgrd
involves: C57BL/6 * CBA * FVB
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
growth/size
• at end-stage, most mice display severe weight loss (>30%)

nervous system
• accumulation of argyrophilic neuronal debris is seen throughout medullary, pontine, and mesencephalic reticular formation up to zona incerta
• signs of reactive gliosis are seen at disease end-stage
• animals not displaying motor abnormalities show ubiquinated neurites in spinal cord
• in mice at end-stage, denervation is observed at neuromuscular synapses
• appears restricted to brainstem and spinal cord
• accumulation of argyrophilic neuronal debris is seen in spinal cord of mice at end-stage of disease; similar amounts are seen between left and right, and lumbar and cervical segments
• loss of motor neurons is detected at disease end-stage

behavior/neurological
• at end-stage, severe locomotor deficits are exhibited
• at end-stage, loss of ability to hang in hanging wire test
• loss of grip strength at end-stage of disease

muscle
• exhibited by most mice (60%) before 2 years of age; onset is observed at 378 to >730 days with end stage reached at >432 to >730 days

cellular
N
• mice do not develop mitochondrial swelling and vacuolization like G1 or G1del mutants (J:134095)

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:134095


Mouse Genome Informatics
tg5
    Tg(Thy1-SOD1*G93A)T3Hgrd/0
involves: C57BL/6 * CBA * FVB
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
behavior/neurological
N
• mice show no clinical or pathological signs of motor abnormalities up to 2 years of age (J:134095)