What is Malonyl-Coa?

Malonyl-Coa is a precursor of fatty acids, which is very important in the regulation of body metabolism of fatty acids. It is necessary for the function of the enzyme known as Malonyl-Coa decarboxylase . The genetic deficiency of this enzyme can lead to a very rare disorder known as the lack of decarboxylase malonyl-Coa. These compounds are necessary to divide as sources of energy, as components of cellular structures such as membranes and as hormones. Excessive amounts of carbohydrates and proteins in the diet can be easily converted to fatty acids. If necessary for energy, fatty acids are easily disintegrated. COA is a coenzyme used in fatty acid synthesis. The basic checkpoint in the greasy acids biosynthesis is the production of Malonyl-Coa. This coenzyme is produced from the addition of carbon dioxide to acetyl-COA. The task of malonyl-Coa in the biosynthesis of fatty acids is to provide growing fatty acid chains with two units of carbon. Acetyl-COA can be used in energy production. Malonyl-Coa decarboxylaseCatalyzes the disintegration of Malonyl-Coa into acetyl-coa and carbon dioxide. Mutation in the gene for this enzyme causes lack of decarboxylase malonyl-coa. This disrupts the metabolism of fatty acids in the body.

With this condition, fatty acids can no longer be converted to energy. This can lead to low blood sugar, also known as hypoglycaemia. The lack of energy from fatty acids is a special problem for heart muscles that rely strongly on these compounds as a source of energy. There is also a build-up of products to collapse fatty acids that contribute to other symptoms.

This disorder is manifested in early childhood, almost always with delayed development. Other symptoms include seizures, vomiting, diarrhea and weak muscle tone. A common weakness is weakening and enlargement of the heart muscle. Experts suggest that low -fat diet could alleviate some symptomy of this disorder.

In order to inherit this lack of enzyme, the person must inherit a copy of the defective gene from each parent. The carrier with only one copy of the defective gene usually shows no symptoms. It requires two copies of defective genes to inherit the lack of decarboxylase. Fortunately, it is an extremely rare disorder. Since 2010, less than 30 cases have been reported.