Mice become paralyzed in one or more limbs and die by 4-5 months of age.
Paralysis is due to loss of motor neurons from the spinal cord.
Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX, Chen W, Zhai P, Sufit RL, Siddique T. 1994. Azzouz M, Leclerc N, Gurney M, Warter JM, Poindron P, Borg J. 1997. Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis.