Malonic Acid Videos
In its purified form, malonic acid is a white, crystalline substance, water-soluble and easily decomposed by heat. Chemically, it is an organic dicarboxylic acid with a methylene group separating the two carbonyl moieties. The diethyl ester of this chemical is used chiefly as an intermediate in the synthesis of barbiturates. It is also used to synthesize vitamins B1 and B6 as well as and numerous other organic compounds. The free acid is rather unstable and has few uses other than being a biochemical diagnostic probe for intermediary metabolism.
The calcium salt of malonic acid occurs in beetroot, but the acid itself is usually prepared by hydrolyzing diethyl malonate. It undergoes the usual reactions of carboxylic acids as well as facile cleavage into acetic acid and carbon dioxide.
Malonic acid is a normal component of human urine, in small quantities, but a genetic disorder called methyl malonic aciduria (also known as malonic aciduria) causes high levels of methyl malonic acid in the blood serum and urine. Patients with this disorder suffer from severe metabolic acidosis and a metabolic block in the vitamin B12 dependent conversion of propionyl CoA to succinyl CoA. In infants, symptoms can include developmental delay, cardiomyopathy, mental retardation, and in its more severe forms, neonatal death.