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.
Similar articles
- 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. - The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.
Mourelatos Z, Gonatas NK, Stieber A, Gurney ME, Dal Canto MC. Mourelatos Z, et al. Proc Natl Acad Sci U S A. 1996 May 28;93(11):5472-7. doi: 10.1073/pnas.93.11.5472. Proc Natl Acad Sci U S A. 1996. PMID: 8643599 Free PMC article. - Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1.
Jaarsma D, Haasdijk ED, Grashorn JA, Hawkins R, van Duijn W, Verspaget HW, London J, Holstege JC. Jaarsma D, et al. Neurobiol Dis. 2000 Dec;7(6 Pt B):623-43. doi: 10.1006/nbdi.2000.0299. Neurobiol Dis. 2000. PMID: 11114261 - Mitochondria in motor nerve terminals: function in health and in mutant superoxide dismutase 1 mouse models of familial ALS.
Barrett EF, Barrett JN, David G. Barrett EF, et al. J Bioenerg Biomembr. 2011 Dec;43(6):581-6. doi: 10.1007/s10863-011-9392-1. J Bioenerg Biomembr. 2011. PMID: 22089637 Free PMC article. Review. - [Superoxide dismutase-1 (SOD-1) gene mutation-dependent mechanisms of neural degeneration in amyotrophic lateral sclerosis].
Iłzecka J. Iłzecka J. Neurol Neurochir Pol. 2001 Mar-Apr;35(3):461-9. Neurol Neurochir Pol. 2001. PMID: 11732268 Review. Polish.
Cited by
- Intranasal delivery of small extracellular vesicles reduces the progress of amyotrophic lateral sclerosis and the overactivation of complement-coagulation cascade and NF-ĸB signaling in SOD1G93A mice.
Zhou J, Li F, Jia B, Wu Z, Huang Z, He M, Weng H, So KF, Qu W, Fu QL, Zhou L. Zhou J, et al. J Nanobiotechnology. 2024 Aug 22;22(1):503. doi: 10.1186/s12951-024-02764-2. J Nanobiotechnology. 2024. PMID: 39174972 Free PMC article. - The prion-like effect and prion-like protein targeting strategy in amyotrophic lateral sclerosis.
Wenzhi Y, Xiangyi L, Dongsheng F. Wenzhi Y, et al. Heliyon. 2024 Jul 22;10(15):e34963. doi: 10.1016/j.heliyon.2024.e34963. eCollection 2024 Aug 15. Heliyon. 2024. PMID: 39170125 Free PMC article. Review. - Inhibition of glycolytic reprogramming suppresses innate immune-mediated inflammation in experimental amyotrophic lateral sclerosis.
Yu L, Wu N, Choi O, Nguyen KD. Yu L, et al. Inflamm Res. 2024 Aug 21. doi: 10.1007/s00011-024-01935-z. Online ahead of print. Inflamm Res. 2024. PMID: 39167140 - HDAC6 inhibition as a mechanism to prevent neurodegeneration in the mSOD1G93A mouse model of ALS.
Phipps AJ, Dwyer S, Collins JM, Kabir F, Atkinson RA, Chowdhury MA, Matthews L, Dixit D, Terry RS, Smith J, Gueven N, Bennett W, Cook AL, King AE, Perry S. Phipps AJ, et al. Heliyon. 2024 Jul 14;10(14):e34587. doi: 10.1016/j.heliyon.2024.e34587. eCollection 2024 Jul 30. Heliyon. 2024. PMID: 39130445 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Molecular Biology Databases
Miscellaneous