Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis
AY Chiu, P Zhai, MC Dal Canto, TM Peters… - Molecular and Cellular …, 1995 - Elsevier
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±7 days of age. Pathological changes
begin by 37 days of age with vacuoles derived from swollen mitochondria accumulating in …
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±7 days of age. Pathological changes
begin by 37 days of age with vacuoles derived from swollen mitochondria accumulating in …