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Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death

Lookup NU author(s): Dr Gillian Borthwick, Dr Margaret Johnson, Professor Pamela Shaw, Emeritus Professor Doug Turnbull


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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.

Publication metadata

Author(s): Borthwick GM; Shaw PJ; Johnson MA; Turnbull DM; Ince PG

Publication type: Article

Publication status: Published

Journal: Annals of Neurology

Year: 1999

Volume: 46

Issue: 5

Pages: 787-790

ISSN (print): 0364-5134

ISSN (electronic): 1531-8249

Publisher: John Wiley & Sons, Inc.


DOI: 10.1002/1531-8249(199911)46:5<787::AID-ANA17>3.0.CO;2-8

PubMed id: 10553999


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