Mitochondria are tiny cellular organelles that harbor their own DNA (mtDNA). They are the major source of energy for all cellular processes, and also play a critical role in proper cellular function and survival. Mitochondrial disease is caused by genetic mutations that may originate either in the cell nucleus or in the mitochondria themselves. The tissues and organs of the body most affected by mitochondrial dysfunction are those that depend heavily on significant amounts of energy for normal function: brain, nerves, muscle, heart, liver, kidney and endocrine glands. The connection between mitochondrial dysfunction and a host of human disease states, particularly those associated with neurological disorders is just beginning to become apparent (see the diagram below).
