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Elevated Body Temperature in Mitochondrial Disorders Results from Uncoupled Oxidative Phosphorylation

Josef Finsterer, Austria

In a recently reported patient with Leigh syndrome due to the mt DNA variant m.9176T>C, a unique phenotypic feature, elevated body temperature, was reported for the first time in adult onset Leigh syndrome. Elevated body temperature was most likely not of central origin but due to uncoupling of the oxidative phosphorylation in muscle cells. It is als conceive a bole that an immunological defect due to the underlying metabolic defects, facilitated the development of chronic infections. In this respect we should know the biochemical defect of the oyidative phosphorylation.

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