• 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 5.5 to 7.5 months of age, mice exhibit axial tremors

• at 5.5 to 7.5 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs

• at 5.5 to 7.5 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice

• eventually mice develop hindlimb paralysis

• at 5.5 to 7.5 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 5.5 to 7.5 months of age