Food is converted in to the body by a complicated process called metabolism. Leucine metabolism is a metabolic disorder caused by an unexpected alteration in enzyme activity. It is a deficientcy of the substance stopping the proper activity of the enzyme or creating a problem with transporting of compounds. If not properly treated, these diseases can be very serious.
According to the type of the disease, you have to switch over to different diet when you are affected with metabolic disorders. For example you should supply the needed leucine, to treat a leucine metabolism disorder and correct your metabolism. Develop your diet plan with the help of a professional dietician and be sure that all your body needs are fully met to correct metabolism.
Disorders of Leucine Metabolism
The three branched amino acids are valine, isoleucine and leucine. When the enzymes in the metabolism of any of three amino acids are not sufficient, organic acids build up in the body.
One of the many diseases of leucine metabolism is “maple syrup urine disease”. High plasma levels of leucine metabolism in the body cause this disease. Clinical test will reveal a body smell, similar to maple syrup. The disease, though very rare, can be serious and may prove fatal if untreated.
Take medical help as you may become lethargic and have vomiting leading to seizures and coma. There should be dietary restrictions from leucine and other branched amino acids. But a small amount of these amino acids can be taken for regular metabolic functions. Isovaleric acidemia is another disease of this branched chain of amino acids.
Dehydrogenetion process is the third step in lecine metabolism. It changes isovalery CoA in to 3-methlcrotonyl CoA. When the process fails and is deficient in 3-methlcrotonyl CoA, it results in “isovalaric academia”. This is also called “sweaty feet” as it gives out a sweat like odour. Sweaty feet may be responsible for vomiting, respiratory problems, hypoglycemia and other serious conditions. If diagnosed for this, a long term control of dietary leucine is required, but you should reinforce with carnitine and glycine, which is important.
Imbalances of metabolism may be set right under medical supervision and nutrition plans. If you suffer from leucine metabolism disorder, your doctor should decide if you need more or less of leucine in your food to see the absorption of balance of nutrients in the body.