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Phenotypes Associated with This Genotype
involves: C57BL/6 * CBA * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-SOD1*G93A)T3Hgrd mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• 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

• 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

• 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

• mice do not develop mitochondrial swelling and vacuolization like G1 or G1del mutants

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

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.01
The Jackson Laboratory