2-Methyl-3-hydroxybutyryl-CoA

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2-Methyl-3-hydroxybutyryl-CoA
2-methyl-3-hydroxybutyryl-CoA.svg
Names
IUPAC name
S-[2-[3-[[4-[[[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] (2S,3S)-3-hydroxy-2-methylbutanethioate
Identifiers
3D model (JSmol)
ChEBI
ChemSpider
KEGG
PubChem CID
  • InChI=1S/C26H44N7O18P3S/c1-13(14(2)34)25(39)55-8-7-28-16(35)5-6-29-23(38)20(37)26(3,4)10-48-54(45,46)51-53(43,44)47-9-15-19(50-52(40,41)42)18(36)24(49-15)33-12-32-17-21(27)30-11-31-22(17)33/h11-15,18-20,24,34,36-37H,5-10H2,1-4H3,(H,28,35)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t13-,14-,15+,18+,19+,20?,24+/m0/s1
    Key: PEKYNTFSOBAABV-SYASONGASA-N
  • C[C@@H]([C@H](C)O)C(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O
Properties
C26H44N7O18P3S
Molar mass 867.7 g/mol
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).

2-Methyl-3-hydroxybutyryl-CoA (2M3HBA [1] ) is an intermediate in isoleucine catabolism.

Related Research Articles

<span class="mw-page-title-main">Isoleucine</span> Chemical compound

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+3 form under biological conditions), an α-carboxylic acid group (which is in the deprotonated −COO− form under biological conditions), and a hydrocarbon side chain with a branch (a central carbon atom bound to three other carbon atoms). It is classified as a non-polar, uncharged (at physiological pH), branched-chain, aliphatic amino acid. It is essential in humans, meaning the body cannot synthesize it. Essential amino acids are necessary in the human diet. In plants isoleucine can be synthesized from threonine and methionine. In plants and bacteria, isoleucine is synthesized from pyruvate employing leucine biosynthesis enzymes. It is encoded by the codons AUU, AUC, and AUA.

<span class="mw-page-title-main">Beta-ketothiolase deficiency</span> Medical condition

Beta-ketothiolase deficiency is a rare, autosomal recessive metabolic disorder in which the body cannot properly process the amino acid isoleucine or the products of lipid breakdown. Along with SCOT deficiency, it belongs to a group of disorders called ketone utilisation disorders.

β-Hydroxybutyric acid Chemical compound

β-Hydroxybutyric acid, also known as 3-hydroxybutyric acid or BHB, is an organic compound and a beta hydroxy acid with the chemical formula CH3CH(OH)CH2CO2H; its conjugate base is β-hydroxybutyrate, also known as 3-hydroxybutyrate. β-Hydroxybutyric acid is a chiral compound with two enantiomers: D-β-hydroxybutyric acid and L-β-hydroxybutyric acid. Its oxidized and polymeric derivatives occur widely in nature. In humans, D-β-hydroxybutyric acid is one of two primary endogenous agonists of hydroxycarboxylic acid receptor 2 (HCA2), a Gi/o-coupled G protein-coupled receptor (GPCR).

<span class="mw-page-title-main">ACADSB</span> Protein-coding gene in the species Homo sapiens

ACADSB is a human gene that encodes short/branched chain specific acyl-CoA dehydrogenase (SBCAD), an enzyme in the acyl CoA dehydrogenase family.

β-Hydroxybutyryl-CoA Chemical compound

β-Hydroxybutyryl-CoA is an intermediate in the fermentation of butyric acid, and in the metabolism of lysine and tryptophan. The L-3-hydroxybutyl-CoA enantiomer is also the second to last intermediate in beta oxidation of even-numbered, straight chain, and saturated fatty acids.

<span class="mw-page-title-main">Ketol-acid reductoisomerase</span> Class of enzymes

In enzymology, a ketol-acid reductoisomerase (EC 1.1.1.86) is an enzyme that catalyzes the chemical reaction

In enzymology, a 3-hydroxy-2-methylbutyryl-CoA dehydrogenase (EC 1.1.1.178) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">3-hydroxyacyl-CoA dehydrogenase</span> Enzyme

In enzymology, a 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">3-Hydroxybutyryl-CoA dehydrogenase</span> Class of enzymes

In enzymology, a 3-hydroxybutyryl-CoA dehydrogenase (EC 1.1.1.157) is an enzyme that catalyzes the chemical reaction

In enzymology, a 2-methylacyl-CoA dehydrogenase is an enzyme that catalyzes the chemical reaction

In enzymology, a 2-methyl-branched-chain-enoyl-CoA reductase (EC 1.3.8.5) is an enzyme that catalyzes the chemical reaction

In enzymology, a 2-oxoisovalerate dehydrogenase (acylating) (EC 1.2.1.25) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">Methylmalonate-semialdehyde dehydrogenase (acylating)</span> Class of enzymes

In enzymology, a methylmalonate-semialdehyde dehydrogenase (acylating) (EC 1.2.1.27) is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">Leucine dehydrogenase</span>

In enzymology, a leucine dehydrogenase (EC 1.4.1.9) is an enzyme that catalyzes the chemical reaction

In enzymology, a 2-acetolactate mutase is an enzyme that catalyzes the chemical reaction

The enzyme dihydroxy-acid dehydratase (EC 4.2.1.9) catalyzes the chemical reaction

In enzymology, a valine-3-methyl-2-oxovalerate transaminase is an enzyme that catalyzes the chemical reaction

<span class="mw-page-title-main">HSD17B10</span> Protein-coding gene in the species Homo sapiens

17-β-Hydroxysteroid dehydrogenase X (HSD10) also known as 3-hydroxyacyl-CoA dehydrogenase type-2 is a mitochondrial enzyme that in humans is encoded by the HSD17B10 gene. Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. Human HSD10 cDNA was cloned from the brain (NM_004493), and the resulting protein, a homotetramer, was first characterized as a short chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD). Active sites of this enzyme can accommodate different substrates; 17β-HSD10 is involved in the oxidation of isoleucine, branched-chain fatty acids, and xenobiotics as well as the metabolism of sex hormones and neuroactive steroids.

<span class="mw-page-title-main">Epicriptine</span> Chemical compound

Epicriptine or beta-dihydroergocryptine is a dopamine agonist of the ergoline class. It constitutes one third of the mixture known as dihydroergocryptine, the other two thirds consisting of alpha-dihydroergocryptine. The alpha differs from the beta form only in the position of a single methyl group, which is a consequence of the biosynthesis in which the proteinogenic amino acid isoleucine is replaced by leucine.

<span class="mw-page-title-main">Sulfometuron methyl</span> Chemical compound

Sulfometuron methyl is an organic compound used as a herbicide. It is classed as a sulfonylurea. It functions via the inhibitition of acetolactate synthase enzyme, which catalyses the first step in biosynthesis of the branched-chain amino acids valine, leucine and isoleucine.

References

  1. ↑ Perez-Cerda, Celia; García-Villoria, Judit; Ofman, Rob; Sala, Pedro Ruiz; Merinero, Begoña; Ramos, Julio; García-Silva, Maria Teresa; Beseler, Beatriz; Dalmau, Jaime; Wanders, Ronald J A.; Ugarte, Magdalena; Ribes, Antonia (January 2005). "2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase (MHBD) Deficiency: An X-linked Inborn Error of Isoleucine Metabolism that May Mimic a Mitochondrial Disease". Pediatric Research . 58 (3): 488–491. doi:10.1203/01.pdr.0000176916.94328.cd. ISSN   1530-0447. PMID   16148061.
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