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In the citric acid cycle, increased fumarate and succinate levels enhance malic enzyme activity and increase the conversion of pyruvate to malate [82], which in turn inhibits the conversion of pyruvate to oxaloacetate when there is an accumulation of acetyl CoA, further increasing the accumulation of malic acid [83]

Characterization of Chorioretinopathy associated with mitochondrial trifunctional protein disorders: long-term follow-up of 21 cases

These findings shed light on potential therapeutic targets for mitigating aNF toxicity

The rate of oxidation slows and this prevents the cells from generating maximum energy from stored fatty acids