Huntington disease is a progressive and incurable genetic condition that affects movement, cognition and behaviour. Brain metabolism in the hereditary disorder is abnormal, with decreased use of glucose. To compensate, researchers believe that branched chain amino acids (BCAA) are used during energy production in the Krebs cycle, meaning BCAA are in short supply. Researchers hypothesised that providing an alternative source of substrates for the Krebs cycle could improve brain metabolism.
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