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Mitochondrial involvement in amyotrophic lateral sclerosis.
Fiona M Menzies1, Paul G Ince, Pamela J Shaw
1Department of Neurology, E Floor, Medical School, Royal Hallamshire Hospital, University of Sheffield, Beech Hill Road, S10 2RX, UK.
Neurochemistry International
|February 19, 2002
Summary
Mitochondrial dysfunction is increasingly implicated in amyotrophic lateral sclerosis (ALS). This review explores evidence linking altered mitochondrial function and structure to motor neuron death in ALS.
Area of Science:
- Neuroscience
- Cell Biology
- Biochemistry
Background:
- The precise mechanisms driving motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remain elusive.
- Early ultrastructural studies hinted at mitochondrial involvement in ALS pathogenesis.
- Recent research provides growing evidence supporting a role for mitochondrial dysfunction in ALS.
Purpose of the Study:
- To review the evidence linking mitochondrial dysfunction to amyotrophic lateral sclerosis (ALS).
- To discuss the relevance of mitochondrial alterations in the context of other ALS etiological hypotheses.
Main Methods:
- Review of existing literature on mitochondrial function and structure in ALS.
- Analysis of studies investigating mitochondrial electron transport chain activity and mitochondrial genome mutations in ALS.
- Examination of research on mitochondrial calcium buffering capacity in ALS.
Main Results:
- Alterations in mitochondrial electron transport chain complex activity have been observed in ALS.
- Mutations in the mitochondrial genome are reported in ALS.
- The calcium buffering role of mitochondria may be impaired in ALS.
Conclusions:
- Mitochondrial dysfunction is a significant feature of amyotrophic lateral sclerosis (ALS).
- Understanding mitochondrial involvement is crucial for elucidating ALS pathogenesis.
- Mitochondrial dysfunction may integrate with other proposed etiological factors in ALS.