Article
Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 1 May 1998
Kong J, Xu Z
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) involves motor neuron degeneration, skeletal muscle atrophy, paralysis, and death. Mutations in Cu,Zn superoxide dismutase (SOD1) are one cause of the disease. Mice transgenic for mutated SOD1 develop symptoms and pathology similar to those in human ALS. To und...
Topics
- Amyotrophic Lateral Sclerosis
- Animals
- Axons
- Disease Progression
- Gene Expression Regulation, Enzymologic
- Humans
- Mice
- Mice, Transgenic
- Mitochondrial Encephalomyopathies
- Motor Neurons
- Mutation
- Nerve Degeneration
- Spinal Cord
- Superoxide Dismutase
- Superoxide Dismutase-1