Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis.

doi:10.1006/mcne.1995.1027

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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. Clini...

Topics

Aging
Amyotrophic Lateral Sclerosis
Animals
Cell Death
Female
Humans
Male
Mice
Mice, Transgenic
Mutation
Nerve Degeneration
Nerve Regeneration
Superoxide Dismutase
Vacuoles

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