methylmalonyl-CoA
Sign in to saveAlso known as Methylmalonyl coenzyme A
Methylmalonyl-CoA is the thioester consisting of coenzyme A linked to methylmalonic acid. It is an important intermediate in the biosynthesis of succinyl-CoA, which plays an essential role in the citric acid cycle.
Wikidata facts
- Mass
- 867.131253
Show 4 more facts
- chemical formula
- C₂₅H₄₀N₇O₁₉P₃S
- isomeric SMILES
- CC(C(=O)O)C(=O)SCCNC(=O)CCNC(=O)[C@@H](C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C2N=CN=C3N)O)OP(=O)(O)O)O
- canonical SMILES
- CC(C(=O)O)C(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OCC1C(C(C(O1)N2C=NC3=C2N=CN=C3N)O)OP(=O)(O)O)O
- found in taxon
- Homo sapiens
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Encyclopedic overview
6 sectionsContents
- Biosynthesis and metabolism
- Vitamin B<sub>12</sub>
- Related diseases
- Methylmalonic Acidemia (MMA)
- Combined malonic and methylmalonic aciduria (CMAMMA)
- References
Methylmalonyl-CoA is the thioester consisting of coenzyme A linked to methylmalonic acid. It is an important intermediate in the biosynthesis of succinyl-CoA, which plays an essential role in the citric acid cycle.
== Biosynthesis and metabolism == left|thumb|Propionate metabolic pathway with L- and D-methylmalonyl-CoA as intermediates. Methylmalonyl-CoA can be synthesized in two ways: From propionyl-CoA: Methylmalonyl-CoA results from the metabolism of fatty acid with an odd number of carbons, of amino acids valine, isoleucine, methionine, threonine or of cholesterol side-chains, forming Propionyl-CoA. The latter is also formed from propionic acid, which bacteria produce in the intestine. Propionyl-CoA and bicarbonate 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:
Excerpted from Wikipedia’s “methylmalonyl-CoA” article, available under the CC BY-SA 4.0 licence.