Which deficiency is associated with lactic acidosis and decreased acetyl CoA levels?

The association of lactic acidosis and decreased acetyl CoA levels with pyruvate dehydrogenase deficiency is rooted in the fundamental role that this enzyme plays in cellular metabolism. Pyruvate dehydrogenase is responsible for catalyzing the conversion of pyruvate into acetyl CoA, which is a critical substrate for the citric acid cycle (Krebs cycle).

In the case of a deficiency of pyruvate dehydrogenase, the conversion of pyruvate to acetyl CoA is impaired. As pyruvate builds up due to this process not running efficiently, it is then shunted toward lactate production rather than entering the citric acid cycle, leading to lactic acidosis. The accumulation of lactate contributes to the lowered pH in the blood, manifesting as lactic acidosis, while simultaneously, the lack of conversion of pyruvate to acetyl CoA results in diminished levels of this vital metabolite.

This metabolic disturbance can severely impair energy production, as acetyl CoA is essential for aerobic respiration through the citric acid cycle, resulting in the clinical consequences observed in pyruvate dehydrogenase deficiency. The condition is often associated with neurological symptoms due to the