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HomeTopics...FatigueMitochondrial Disease
Mitochondrial DiseaseMitochondrial disease is very difficult to diagnose because there is no defining symptom to the illness. Furthermore, mitochondrial disease can take many forms, depending on the severity of the disease and the location that is affected.
An estimated 50,000 Americans suffer from mitochondrial disease.
What are Mitochondria?The mitochondria are the power plants of the cell because they are responsible for processing oxygen and converting substances from the foods we eat into energy for cell function. Utilizing the oxygen and certain substances, the mitochondria produce energy in the form of adenosine triphosphate (ATP), which is then broken down and used by the cell.
The number of mitochondria in each cell varies anywhere from a few to a few thousand. Cells that require more energy (liver, muscle, heart) will have more mitochondria.
 Once Mitochondrial Disease Sets in…The process of converting oxygen and food into energy requires hundreds of chemical reactions. If any one reaction does not run smoothly or efficiently, the production of energy is severely hampered. It is important to remember that many of the intermediate products (food that has not completely been converted to energy) are toxic to the cell. If the reactions don't run properly, the intermediate products may build up in the cell and hamper other reactions or cause damage to the mitochondria and the cell.
Mitochondrial disease may affect the mitochondria in numerous ways: one of those ways being the many chemical reactions that take place. Incomplete reactions mean no energy production. No energy production means the cell cannot operate properly, and may eventually result in the death of the cell. If a large number of cells, or important cells, are affected, you can see how this can become a serious problem.
Causes of Mitochondrial DiseaseFor most people, mitochondrial disease is inherited. While other possibilities exist (such as mitochondrial toxins), the biggest cause of mitochondrial disease is inheritance.
One of the most interesting things about mitochondria is the presence of DNA within the mitochondria. While most people are familiar with the DNA in the cell nucleus, the DNA in mitochondria act a bit differently. While the DNA of the cell receives one copy from the mother and one copy from the father, all (or nearly all) of the mitochondrial DNA is inherited from the mother. This is due to the sheer size of the egg cell, which is significantly larger than the sperm cell. Furthermore, while a regular cell contains two copies of DNA, mitochondria may hold anywhere from five to ten copies of DNA at one time. If a majority of the DNA is defective, it can quickly become a big problem. Another possible cause for mitochondrial disease may occur during the replication of mitochondrial DNA. While the cell's DNA has an error checking capability, mitochondrial DNA does not. This means that the DNA may be replicated with errors. Furthermore, the enzymes that control the replication of mitochondrial DNA may also be defective. If that is the case, mutations (and problems) in the mitochondrial DNA may be a common occurrence.
Symptoms of Mitochondrial DiseaseThe symptoms of mitochondrial disease vary with the severity of the disease and the location of the disease. If the disease is in the brain, you will notice a marked reduction in mental functions. If it is in the muscles, you'll notice muscle weakness and poor coordination. Some of the more common symptoms include:
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