Mouse Genome Informatics
involves: C3H/HeJ * C57BL/6J
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• numerous ubiquitin-immunoreactive, Thioflavin-S positive fibrillar protein aggregates resembling hyaline inclusions are seen in the spinal cord, ventral midbrain, and the brain stem, with fewer in the cerebellum
• at late stages
• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem
• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons

• at 6 to 8 months of age, mice exhibit axial tremors
• at 6 to 8 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs
• at 6 to 8 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice
• eventually mice develop hindlimb paralysis

• at 6 to 8 months of age, mice exhibit muscle wasting particularly along the flanks
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

• motor neurons exhibit mitochondrial degradation

• progressive

• at 6 to 8 months of age

Mouse Models of Human Disease
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69178 , J:119631