Physicians, and most biologists, are taught that mitochondria are monomorphic little sausages in our cells that make adenosine triphosphate (ATP).
In reality, mitochondria take on many different shapes and subserve a great number of different metabolic functions. Each mitochondrion’s shape is characteristic of the specialized cell in which it resides.
In the liver, mitochondria are specialized to detoxify ammonia in the urea cycle. Mitochondria are also required for cholesterol metabolism, for estrogen and testosterone synthesis, for neurotransmitter metabolism, and for free radical production and detoxification.
They do all this in addition to breaking down (oxidizing) the fat, protein, and carbohydrates we eat and drink.
Because mitochondria perform so many different functions in different tissues, there are literally hundreds of different mitochondrial diseases. Even physicians working in highly specialized referral centers who see dozens of cases of mitochondrial disease every year are struck by the great diversity of signs and symptoms.
Excerpted from “The Spectrum of Mitochondrial Disease”