Mouse Genome Informatics
involves: C57BL/6 * CBA * FVB
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
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:134095