Methylmalonyl-CoA

Methylmalonyl-CoA is a molecule that plays a crucial role in human metabolism. It is involved in the breakdown and use of proteins and fats in the body. Methylmalonyl-CoA is converted into succinyl-CoA, a key component of the citric acid cycle, through a reaction catalyzed by the enzyme methylmalonyl-CoA mutase.

Structure and Function[edit | edit source]

Methylmalonyl-CoA is a derivative of coenzyme A, a molecule that is essential for many biochemical reactions in the body. The structure of methylmalonyl-CoA includes a carboxyl group, a thiol group, and a methyl group, which are attached to a ribose sugar and an adenine base.

The primary function of methylmalonyl-CoA is to facilitate the breakdown of certain amino acids and fatty acids. This process is crucial for the production of energy in the body.

Role in Metabolism[edit | edit source]

In the process of metabolism, methylmalonyl-CoA is converted into succinyl-CoA, a molecule that is a key component of the citric acid cycle. This conversion is catalyzed by the enzyme methylmalonyl-CoA mutase, which requires vitamin B12 (cobalamin) as a cofactor.

If there is a deficiency of methylmalonyl-CoA mutase or vitamin B12, the conversion of methylmalonyl-CoA to succinyl-CoA is impaired. This can lead to a buildup of methylmalonyl-CoA and related compounds in the body, a condition known as methylmalonic acidemia.

Methylmalonic Acidemia[edit | edit source]

Methylmalonic acidemia is a rare genetic disorder that affects the body's ability to break down certain proteins and fats. Symptoms can include vomiting, dehydration, weakness, and developmental delay. Treatment typically involves a low-protein diet and supplements of vitamin B12.