Methylmalonsäure
Methylmalonic acid is a dicarboxylic acid with the chemical formula C4H6O4. It is an organic compound that plays a role in metabolism. Specifically, methylmalonic acid is an intermediate in the metabolism of certain amino acids, namely valine, isoleucine, threonine, and methionine. It is also involved in the breakdown of odd-chain fatty acids. The body typically converts methylmalonic acid into succinyl-CoA, which can then enter the citric acid cycle for energy production. This conversion is facilitated by an enzyme called methylmalonyl-CoA mutase, which requires vitamin B12 (cobalamin) as a cofactor.
Elevated levels of methylmalonic acid in the blood or urine can be an indicator of several medical