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L-2-Hydroxyglutaric aciduria (L-2-HGA) is caused by mutations in the gene encoding L-2-hydroxyglutarate dehydrogenase (LHGDH), leading to the accumulation of L-2-HG in the brain and biological fluids, which is the biochemical hallmark of L-2-HGA [111, 112]

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CACT and CPT II are bi-directionally catalytic, which means that LCACs can be generated within the mitochondria and exported into the cytosol and, eventually, into the circulation (Noland et al., 2009

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