(''R'')-2,3-Dihydroxy-isovalerate, also known as α,β-dihydroxyisovalerate, is an important intermediate in the

biosynthesis Biosynthesis, i.e., chemical synthesis occurring in biological contexts, is a term most often referring to multi-step, enzyme-Catalysis, catalyzed processes where chemical substances absorbed as nutrients (or previously converted through biosynthe ...

branched-chain amino acids A branched-chain amino acid (BCAA) is an amino acid having an aliphatic side-chain with a branch (a central carbon atom bound to three or more carbon atoms). Among the proteinogenic amino acids, there are three BCAAs: leucine, isoleucine, and va ...

, particularly

valine Valine (symbol Val or V) is an α-amino acid that is used in the biosynthesis of proteins. It contains an α- amino group (which is in the protonated −NH3+ form under biological conditions), an α- carboxylic acid group (which is in the deproton ...

leucine Leucine (symbol Leu or L) is an essential amino acid that is used in the biosynthesis of proteins. Leucine is an α-amino acid, meaning it contains an α-amino group (which is in the protonated −NH3+ form under biological conditions), an α-Car ...

, and

isoleucine Isoleucine (symbol Ile or I) is an α-amino acid that is used in the biosynthesis of proteins. It contains an α-amino group (which is in the protonated −NH form under biological conditions), an α-carboxylic acid group (which is in the depro ...

. This organic compound plays a crucial role in several metabolic pathways, including the superpathway of leucine, valine, and isoleucine biosynthesis, as well as pantothenate and

coenzyme A Coenzyme A (CoA, SHCoA, CoASH) is a coenzyme, notable for its role in the Fatty acid metabolism#Synthesis, synthesis and Fatty acid metabolism#.CE.B2-Oxidation, oxidation of fatty acids, and the oxidation of pyruvic acid, pyruvate in the citric ac ...

(CoA) biosynthesis. It is produced from 3-hydroxy-3-methyl-2-oxobutanoic acid by the enzyme

ketol-acid reductoisomerase In enzymology, a ketol-acid reductoisomerase () is an enzyme that catalyzes the chemical reaction :(R)-2,3-dihydroxy-3-methylbutanoate + NADP+ \rightleftharpoons (S)-2-hydroxy-2-methyl-3-oxobutanoate + NADPH + H+ Thus, the two substrates of thi ...

(EC 1.1.1.86) and is subsequently converted to 2-oxoisovalerate by the enzyme

dihydroxy-acid dehydratase The enzyme dihydroxy-acid dehydratase () catalyzes the chemical reaction :2,3-dihydroxy-3-methylbutanoate \rightleftharpoons 3-methyl-2-oxobutanoate + H2O This enzyme participates in valine, leucine and isoleucine biosynthesis and pantothenate a ...

(EC 4.2.1.9).

References

{{DEFAULTSORT:Dihydroxy-isovalerate, (R)-2, 3- Branched-chain amino acids Alpha hydroxy acids