PerspectiveMitochondria

Mitochondrial disease therapy from thin air?

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Science  01 Apr 2016:
Vol. 352, Issue 6281, pp. 31-32
DOI: 10.1126/science.aaf5248

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Summary

The mitochondrial oxidative phosphorylation (OXPHOS) system supplies most of the adenosine 59-triphosphate (ATP) in the human body. Defects in this system are among the most common genetic metabolic diseases, and many are associated with fatal, early-onset neurodegenerative conditions for which there are no effective treatments. On page 54 of this issue, Jain et al. (1) propose that restricting the oxygen supply to patients, who already have a defect in aerobic ATP synthesis, may be a form of therapy. This seems counterintuitive. How did the authors reach that conclusion?