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L-carnitine is a natural component of the body and is involved in the transport of fatty acids, supporting processes related to energy metabolism

doi: 10.1038/nri.2016.70 138 SerbuleaVUpchurchCMAhernKWBoriesGVoigtPDeWeeseDEet al

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44, 77 Methymalonic acid, a metabolite of PPA, results in abnormal mitochondrial morphology, 78 tissue specific ETC dysfunction, 79 inhibition of the complex I and II function when interacting with complex III in the brain 80 and reductions in reduced glutathione, 78 similar to the patient cohort presented and the PPA animal model

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