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Brief Communication
Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death
Article first published online: 17 MAY 2001
DOI: 10.1002/1531-8249(199911)46:5<787::AID-ANA17>3.0.CO;2-8
Copyright © 1999 American Neurological Association
1531-8249/asset/cover.gif?v=1&s=685ec69724c5ed4c8847ce939e70ceb45065856f "Annals of Neurology")
Additional Information
How to Cite
Borthwick, G. M., Johnson, M. A., Ince, P. G., Shaw, P. J. and Turnbull, D. M. (1999), Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death. Ann Neurol., 46: 787–790. doi: 10.1002/1531-8249(199911)46:5<787::AID-ANA17>3.0.CO;2-8
Publication History
- Issue published online: 17 MAY 2001
- Article first published online: 17 MAY 2001
- Manuscript Revised: 16 JUL 1999
- Manuscript Accepted: 16 JUL 1999
- Manuscript Received: 11 MAR 1999
Funded by
- Wellcome Trust, UK
- Abstract
- References
- Cited By
Abstract
The mechanism of selective loss of motor neurons in amyotrophic lateral sclerosis (ALS) has not been clarified. Mitochondrial pathology is present in central nervous system tissue from ALS cases and occurs as an early event in a mouse model of ALS. We demonstrate that, in sporadic ALS, there is a selective decrease in the activity of the mitochondrial DNA–encoded enzyme cytochrome c oxidase in human spinal cord motor neurons. We propose that this may not only be important in neuronal cell death but could well be caused by oxidative damage to mitochondrial DNA leading to the accumulation of mitochondrial DNA mutations.