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Abnormalities in Energy Metabolism Disease Mechanism III
The altered huntingtin protein has been found to cause abnormalities in energy metabolism - the process by which cells make energy. The altered huntingtin protein interacts with key proteins involved in energy production and causes damage to the mitochondria – the site of energy production in cells. Cells continually produce energy in the form of molecules known as ATP (for “adenosine triphosphate”). ATP is then used by the cell to perform various functions. The abnormality in energy metabolism in HD cells results in lower amounts of ATP available in cells, and makes the cells more susceptible to damage by toxic compounds. Drugs and supplements that increase the amount of energy available in cells are being looked into as possible candidates for treating HD. An understanding of how huntingtin affects energy metabolism is important to be able to understand how these drugs improve energy production in the cell. (Click here for more information on Huntingtin and Energy Metabolism.)
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