METHYLMALONYL COENZYME A LITHIUM

Uses

Methylmalonyl coenzyme A (CoA) has been used in the preparation of the calibration curve in ultra-performance liquid chromatography-mass spectrometry (UPLC-MS/MS). It has also been used in the measurement of methylmalonyl-CoA mutase and methylmalonyl-CoA epimerase activity.

Biochem/physiol Actions

Methylmalonyl coenzyme A (CoA) is a catabolite of odd-chain fatty acids, cholesterol and few amino acids like valine, isoleucine, methionine, threonine. It is produced by the activity of the enzyme propionyl-CoA carboxylase(PCC). Methylmalonyl coenzyme A (CoA) is converted to succinyl-CoA by the methylmalonyl-CoA mutase (MCM) and vitamin B12 as the cofactor. Either a low MCM activity or an altered metabolism of vitamin B12 causes the inherited metabolic disease methylmalonic acidemia (MMA).