Article
Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis.
Molecular and cellular neurosciences - 1 Aug 1995
Chiu A Y, Zhai P, Dal Canto M C, Peters T M, Kwon Y W, Prattis S M, Gurney M E
Abstract excerpt
The mutation gly93-->ala of Cu,Zn superoxide dismutase (SOD) is found in patients with familial amyotrophic lateral sclerosis and causes motor neuron disease when expressed in transgenic mice. The progression of clinical and pathological disease was studied in a line of mice designated G1H. Clinical disease started at 91 +/- 14 days of age with fine shaking of the limbs, followed by paralysis and death by 136 +/-...
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