Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation - PubMed (original) (raw)
. 1994 Jun 17;264(5166):1772-5.
doi: 10.1126/science.8209258.
Affiliations
- PMID: 8209258
- DOI: 10.1126/science.8209258
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation
M E Gurney et al. Science. 1994.
Erratum in
- Science 1995 Jul 14;269(5221):149
Abstract
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 activity--caused motor neuron disease in transgenic mice. The mice became paralyzed in one or more limbs as a result of motor neuron loss from the spinal cord and died by 5 to 6 months of age. The results show that dominant, gain-of-function mutations in SOD contribute to the pathogenesis of familial ALS.
Comment in
- Mutant mice, Cu,Zn superoxide dismutase, and motor neuron degeneration.
McCord JM. McCord JM. Science. 1994 Dec 2;266(5190):1586-7. Science. 1994. PMID: 7985031 No abstract available. - Mouse model found for ALS.
Marx J. Marx J. Science. 1994 Jun 17;264(5166):1663-4. doi: 10.1126/science.8209242. Science. 1994. PMID: 8209242 No abstract available.
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