About 10-20% of patients with Leigh Syndrome, a mitochondrial disease, have a mutation in MT-ATP6, a gene that codes for ATP synthase. These patients often experience high levels of _____ in their cells due to an increase in levels of pyruvate that are unable to convert to acetyl-CoA.

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When pyruvate cannot be converted into acetyl-CoA due to mitochondrial dysfunction, as seen in Leigh Syndrome, it is instead converted into lactate. This process helps regenerate NAD+ from NADH, allowing glycolysis to continue producing ATP in the absence of efficient mitochondrial ATP production.

About 10-20% of Patients with Leigh Syndrome, a Mitochondrial Disease