This page is here to help you understand a little more about one of the most complex and important parts of the cell. It is not intended to be a very technical/accurate description by any means.

This page is for those who would like to know a little more about the mitochondria and what it does. There will be some basic biochemistry discussed here too. The mitochondria is responsible for producing the energy in each cell. Though it is small in size, it is perhaps the most important portion of each cell in our body with the exception of the sperm cell which has no mitochondria in it.

Three things must occur in order for the mitochondria to do its work. If there are any problems in any one or combination of steps in this process, then the person will have mitochondrial disease. The three steps are as follows:

1. The body must be able to deliver the food to the body. Food can be either protein, fat, or carbohydrates (sugars). This is no easy task, and for those who have problems with this step can have any number of problems because of the build up of the particular food.
   

2. Assuming that the food can get into the mitochondria, then many complex things happen to break down the food into usable pieces. Much of this work is done in a series of chemical reactions known as the Krebs Cycle. The second part of this stage is to try to assemble the pieces into usable parts. This takes place in the electron transport mechanism or Respiratory Chain. Many people with mitochondrial disease have problems with this stage. (Rhys is Complex I and III deficient - see the diagrams at the bottom of the page).
   

3. The last step consists of using the pieces to make the energy. Some people are unable to do this.

Assuming that you can understand this (not too easy a task), there is one last thing that you should know. How sick a person is depends on how many "bad" mitochondria are in each cell. There is no fixed number of mitochondria in each cell, and if the number of good mitochondria are more than the defective mitochondria, then symptoms may not be as severe.

To summarize the above, the body must be able to get food into the mitochondria in the first step. The food can either be protein, fat, or carbohydrates (sugars). Assuming that the body can get the food there, many complex reactions occur inside the mitochondria to break the food down into usable pieces. Lastly, these pieces are used to make the energy

Now for the diagrams. For those of you who are familiar with mitochondrial disease, note that when a muscle biopsy is frozen for testing purposes, the inner membrane of the mitochondria is permanently damaged. This makes the mitochondria useless for testing how it works (important if trying to determine if a person has too low a level of any of the Respiratory Chain Complexes).