Pyruvate dehydrogenase complex deficiency
Definition
Pyruvate dehydrogenase complex deficiency (PDHA) is a genetic disorder that results in a malfunctioning of the Krebs, or tricarboxylic acid (TCA), cycle. It is sex-linked and appears to be a dominant trait.
Description
PDHA is one of the most common of the genetic disorders that cause abnormalities of mitochondrial metabolism. The mitochondria are the organelles inside cells that are reponsible for energy production and respiration at the cellular level. One of the most important processes in the mitochondria is the TCA cycle (also known as the Krebs cycle). The TCA cycle produces the majority of the ATP (chemical energy) necessary for maintenance (homeostasis) of the cell. The production of...
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