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Methylmalonyl-CoA is the thioester consisting of
Methylmalonyl-CoA results from the
coenzyme A
Coenzyme A (CoA, SHCoA, CoASH) is a coenzyme, notable for its role in the synthesis and oxidation of fatty acids, and the oxidation of pyruvate in the citric acid cycle. All genomes sequenced to date encode enzymes that use coenzyme A as a subs ...
linked to methylmalonic acid. It is an important intermediate in the biosynthesis
Biosynthesis is a multi-step, enzyme-catalyzed process where substrates are converted into more complex products in living organisms. In biosynthesis, simple compounds are modified, converted into other compounds, or joined to form macromolecul ...
of succinyl-CoA
Succinyl-coenzyme A, abbreviated as succinyl-CoA () or SucCoA, is a thioester of succinic acid and coenzyme A.
Sources
It is an important intermediate in the citric acid cycle, where it is synthesized from α-ketoglutarate by α-ketoglutarate d ...
, which plays an essential role in the tricarboxylic acid cycle (aka the Citric Acid Cycle, or Krebs Cycle). The compound is sometimes referred to as "methylmalyl-CoA".
Biosynthesis and metabolism
Methylmalonyl-CoA results from the metabolism
Metabolism (, from el, μεταβολή ''metabolē'', "change") is the set of life-sustaining chemical reactions in organisms. The three main functions of metabolism are: the conversion of the energy in food to energy available to run ...
of fatty acid
In chemistry, particularly in biochemistry, a fatty acid is a carboxylic acid with an aliphatic chain, which is either saturated or unsaturated. Most naturally occurring fatty acids have an unbranched chain of an even number of carbon atoms, f ...
with an odd number
In mathematics, parity is the property of an integer of whether it is even or odd. An integer is even if it is a multiple of two, and odd if it is not.. For example, −4, 0, 82 are even because
\begin
-2 \cdot 2 &= -4 \\
0 \cdot 2 &= 0 \\
41 ...
of carbons or from cholesterol side-chains, forming Propionyl-CoA. Propionyl-CoA and bicarbonate
In inorganic chemistry, bicarbonate (IUPAC-recommended nomenclature: hydrogencarbonate) is an intermediate form in the deprotonation of carbonic acid. It is a polyatomic anion with the chemical formula .
Bicarbonate serves a crucial biochemi ...
are converted to Methylmalonyl-CoA by the enzyme propionyl-CoA Carboxylase. It then is converted into succinyl-CoA by methylmalonyl-CoA mutase (MUT). This reaction is a reversible isomerization. In this way, the compound enters the Citric Acid Cycle. The following diagram demonstrates the aforementioned reaction:
Propionyl CoA + Bicarbonate → Methylmalonyl CoA → Succinyl CoA
Vitamin B12
Vitamin B12 plays an integral role in this reaction. Coenzyme B12 ( adenosyl-cobalamin) is anorganometallic
Organometallic chemistry is the study of organometallic compounds, chemical compounds containing at least one chemical bond between a carbon atom of an organic molecule and a metal, including alkali, alkaline earth, and transition metals, and s ...
form of Vitamin B12 and serves as the cofactor of Methylmalonyl-CoA mutase, which is an essential enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products ...
in the human body. The transformation of Methylmalonyl-CoA to Succinyl-CoA by this enzyme is a radical reaction.
Related diseases
Methylmalonic Acidemia (MMA)
This disease occurs when methylmalonyl-CoA mutase is unable to isomerize sufficient amounts of methylmalonyl-CoA into succinyl-CoA. This causes a buildup of propionic and/or methylmalonic acid, which has effects on infants ranging from severe brain damage to death. The disease is linked to Vitamin B12, which is the metabolic precursor to methylmalonyl-CoA mutase.References
Thioesters of coenzyme A {{Microbiology-stub