Article
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.
Science (New York, N.Y.) - 17 Jun 1994
Gurney M E, Pu H, Chiu A Y, Dal Canto M C, Polchow C Y, Alexander D D, Caliendo J, Hentati A, Kwon Y W, Deng H X
Abstract excerpt
Mutations of human Cu,Zn superoxide dismutase (SOD) are found in about 20 percent of patients with familial amyotrophic lateral sclerosis (ALS). Expression of high levels of human SOD containing a substitution of glycine to alanine at position 93--a change that has little effect on enzyme activit...
Topics
- Amyotrophic Lateral Sclerosis
- Animals
- Brain
- Disease Models, Animal
- Female
- Humans
- Male
- Mice
- Mice, Inbred C57BL
- Mice, Transgenic
- Motor Endplate
- Motor Neuron Disease
- Motor Neurons
- Muscles
- Mutation
- Pedigree
- Spinal Cord
- Superoxide Dismutase