Fatty Acid Oxidation Disorders

What are Fatty Acid Oxidation Disorders?

People who have Fatty Acid Oxidation Disorders are unable to convert fat into energy in the cells.
How are carbohydrates and fats converted into the energy we need to exercise?
Carbohydrates and fat are stored in different ways, and are broken down to provide energy by different processes. Carbohydrates are broken down into glucose that is then stored as glycogen. Glycogen is stored in the muscles, heart, liver and brain. When we exercise intensely in short bursts, the glycogen is converted back into glucose and then into a substance called pyruvate. The pyruvate then enters the mitochondria (the ‘powerhouses’ in cells whose major function is to produce energy units known at ATP). If the exercise is strenuous it can overload the system which cannot function fast enough to convert all the pyruvate into energy via the mitochondria. As a result, some of the pyruvate is turned into lactic acid. Fat is more of a storage fuel. It is stored in various places such as under the skin. We usually use fat for energy during prolonged exercise. The fat is brought out of the tissue where it is stored, goes into the bloodstream and then into muscle, and finally continues into the mitochondria. Once inside the mitochondria, the fats are broken down into carbon dioxide, water and energy (ATP) during a process called fatty acid oxidation.
Related Organizations
The Adult Metabolic Diseases Clinic
Vancouver General Hospital
Diamond Health Care Centre
4th Floor, 2775 Laurel Street
Vancouver, BC
V5Z 1M9
(604) 875-5965
The National Organization for Rare Disorders, Inc.
P.O. Box 8923
New Fairfield, CT, 06812-8923
Web site: www.rarediseases.org
Phone: 1-800-999-6673
Aboriginal Patient Navigators
Aboriginal Patient Navigators help aboriginal patients through the health system and offer support in other social health determinant areas such as housing and income.
-When you are booking your appointment, please ask if you’d like us to arrange for a Patient Navigator to come with you to your next appointment
Phone: 604-875-5600 ext 63292; Toll-free: 1-877-875-1131
Fatty Oxidation Disorders (FOD) Family Support Group
Web site: www.fodsupport.org

Useful Resources

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Medium-chain acyl-CoA dehydrogenase
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Very long-chain acyl-CoA dehydrogenase deficiency