methylmalonylKS
Methylmalonyl-CoA (MM-CoA) is an intermediate in the metabolism of odd-chain fatty acids and certain amino acids. It is derived from the breakdown of propionyl-CoA, which is produced from the metabolism of isoleucine, methionine, and valine. The conversion of propionyl-CoA to MM-CoA is catalyzed by the enzyme propionyl-CoA carboxylase (PCC), which requires biotin as a cofactor.
MM-CoA is then converted to succinyl-CoA, a key intermediate in the citric acid cycle (Krebs cycle), by
Deficiencies in the enzymes involved in the metabolism of MM-CoA, such as PCC and MCM, can lead
In summary, methylmalonyl-CoA is a crucial intermediate in the metabolism of odd-chain fatty acids and certain