Methylmalonic acid

Methylmalonic acid (MMA) is a dicarboxylic acid that is a vital intermediate in the metabolism of fat and protein. It is a substance that is produced when proteins, specifically those containing the amino acid methionine, are broken down in the body.

Chemistry[edit | edit source]

Methylmalonic acid is a chiral compound with two enantiomers, (R)- and (S)-methylmalonic acid. It is a colorless, crystalline solid that is soluble in water. The chemical formula for methylmalonic acid is C4H6O4.

Biosynthesis[edit | edit source]

Methylmalonic acid is produced in the body during the metabolism of certain amino acids and odd-chain fatty acids. The process begins with the conversion of these substances into propionyl-CoA, which is then converted into methylmalonyl-CoA by the enzyme propionyl-CoA carboxylase. Methylmalonyl-CoA is then converted into succinyl-CoA, a key intermediate in the citric acid cycle, by the enzyme methylmalonyl-CoA mutase.

Clinical significance[edit | edit source]

Elevated levels of methylmalonic acid in the blood, a condition known as methylmalonic acidemia, can be a sign of several different health problems, including vitamin B12 deficiency, kidney disease, and certain genetic disorders. Methylmalonic acidemia is a serious condition that can lead to developmental delays, failure to thrive, and other health problems if not treated promptly.