• Thumbnail for Methylglutaconyl-CoA hydratase
    3-Methylglutaconyl-CoA hydratase, also known as MG-CoA hydratase and AUH, is an enzyme (EC 4.2.1.18) encoded by the AUH gene on chromosome 19. It is a...
    7 KB (1,415 words) - 14:40, 27 July 2022
  • Thumbnail for Enoyl-CoA hydratase
    Enoyl-CoA hydratase (ECH) or crotonase is an enzyme EC 4.2.1.17 that hydrates the double bond between the second and third carbons on 2-trans/cis-enoyl-CoA:...
    5 KB (1,036 words) - 16:50, 31 December 2023
  • Thumbnail for 3-Methylglutaconyl-CoA
    leucine. It is metabolized into HMG-CoA. Methylcrotonyl-CoA carboxylase Methylglutaconyl-CoA hydratase Wilson JM, Fitschen PJ, Campbell B, Wilson GJ, Zanchi...
    4 KB (644 words) - 17:57, 4 October 2023
  • Thumbnail for Acetyl-CoA
    catalyzed by four respective enzymes, namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces...
    22 KB (2,091 words) - 16:58, 17 April 2024
  • Thumbnail for HMG-CoA
    precursors are β-methylglutaconyl-CoA (MG-CoA) and β-hydroxy β-methylbutyryl-CoA (HMB-CoA). HMG-CoA reductase catalyzes the conversion of HMG-CoA to mevalonic...
    8 KB (959 words) - 23:27, 8 December 2021
  • Thumbnail for Β-Hydroxy β-methylbutyric acid
    subsequently converted to HMG-CoA by methylglutaconyl-CoA hydratase. HMG-CoA is then cleaved into acetyl-CoA and acetoacetate by HMG-CoA lyase or used in the production...
    106 KB (9,982 words) - 15:37, 7 January 2024
  • Thumbnail for Leucine
    subsequently converted to HMG-CoA by methylglutaconyl-CoA hydratase. HMG-CoA is then cleaved into acetyl-CoA and acetoacetate by HMG-CoA lyase or used in the production...
    27 KB (4,136 words) - 02:03, 6 April 2024
  • Thumbnail for 3-Methylglutaconic aciduria
    3-Methylglutaconic aciduria type I, 3-Methylglutaconic acidemia or 3-Methylglutaconyl-CoA Hydratase Deficiency Mutations in the AUH gene cause 3-methylglutaconic...
    8 KB (693 words) - 09:18, 5 October 2023
  • CoA, to form 3-methylglutaconyl CoA. MCC is covalently modified and inhibited by intermediates of leucine catabolism including 3-methylglutaconyl-CoA...
    9 KB (1,329 words) - 07:55, 18 August 2023
  • Thumbnail for Beta-Hydroxy beta-methylbutyryl-CoA
    diverts methylcrotonyl CoA to 3-hydroxyisovaleryl CoA in a reaction catalyzed by enoyl-CoA hydratase (22, 23). 3-Hydroxyisovaleryl CoA accumulation can inhibit...
    3 KB (679 words) - 16:52, 28 October 2023
  • Thumbnail for Β-Hydroxybutyryl-CoA
    Yundong (2017-09-07). "Chromodomain Protein CDYL Acts as a Crotonyl-CoA Hydratase to Regulate Histone Crotonylation and Spermatogenesis". Molecular Cell...
    4 KB (260 words) - 09:49, 4 September 2023
  • Thumbnail for William Nyhan
    metabolism disorders, among them 4-hydroxybutyric aciduria, 3-methylglutaconyl-Co A hydratase deficiency, multiple carboxylase deficiency, methylmalonic...
    3 KB (266 words) - 13:54, 6 December 2023
  • Thumbnail for Β-Hydroxybutyric acid
    diverts methylcrotonyl CoA to 3-hydroxyisovaleryl CoA in a reaction catalyzed by enoyl-CoA hydratase (22, 23). 3-Hydroxyisovaleryl CoA accumulation can inhibit...
    14 KB (1,416 words) - 08:15, 7 January 2024
  • butyric aciduria Isobutyryl-CoA dehydrogenase deficiency 2-Methylbutyryl-CoA dehydrogenase deficiency 3-Methylglutaconyl-CoA hydratase deficiency Glutaric acidemia...
    7 KB (716 words) - 23:56, 13 September 2023
  • methylcrotonic aciduria 3 methylglutaconyl coa hydratase deficiency 3-hydroxy 3-methyl glutaryl-coa lyase deficiency 3-hydroxyacyl-coa dehydrogenase deficiency...
    3 KB (307 words) - 03:36, 11 December 2023
  • 19, threonine ammonia-lyase EC 4.2.1.17: enoyl-CoA hydratase EC 4.2.1.18: methylglutaconyl-CoA hydratase EC 4.2.1.19: imidazoleglycerol-phosphate dehydratase...
    62 KB (7,931 words) - 19:04, 4 January 2024
  • Thumbnail for Isovaleryl-CoA dehydrogenase
    enzymology, an isovaleryl-CoA dehydrogenase (EC 1.3.8.4) is an enzyme that catalyzes the chemical reaction 3-methylbutanoyl-CoA + acceptor ⇌ {\displaystyle...
    3 KB (684 words) - 14:37, 26 August 2023
  • Thumbnail for Ferrichrome A
    step involves the conversion of HMG-CoA to methyl glutaconyl-CoA via enoyl-CoA hydratase (Fer4). Methyl glutaconyl-CoA is then coupled with hydroxy ornithine...
    5 KB (482 words) - 04:22, 3 July 2022
  • Thumbnail for Methylcrotonyl-CoA
    3-Methylcrotonyl-CoA (β-Methylcrotonyl-CoA or MC-CoA) is an intermediate in the metabolism of leucine. It is found in mitochondria, where it is formed...
    5 KB (709 words) - 14:32, 3 December 2023
  • Thumbnail for Isovaleryl-CoA
    Isovaleryl-coenzyme A, also known as isovaleryl-CoA, is an intermediate in the metabolism of branched-chain amino acids. Isovaleryl coenzyme A dehydrogenase...
    5 KB (636 words) - 00:08, 3 May 2023
  • Thumbnail for Α-Ketoisocaproic acid
    cofactors present, including cholesterol, acetyl-CoA, isovaleryl-CoA, and other biological molecules. Isovaleryl-CoA is the main compound synthesized from ɑ-KIC...
    12 KB (1,572 words) - 20:02, 4 January 2024
  • Thumbnail for Cysteine sulfinic acid
    containing this residue are found at the active site of some nitrile hydratases. Akter, Salma; Fu, Ling; Jung, Youngeun; Conte, Mauro Lo; Lawson, J. Reed;...
    4 KB (304 words) - 09:26, 15 April 2024
  • Thumbnail for Urocanic acid
    of ammonium. In the liver, urocanic acid is transformed by urocanate hydratase (or urocanase) to 4-imidazolone-5-propionic acid and subsequently to glutamic...
    7 KB (671 words) - 04:03, 25 March 2024
  • Thumbnail for Imidazol-4-one-5-propionic acid
    relevant to type II diabetes. Formiminoglutamic acid Urocanate Urocanate hydratase Hassall, H.; Greenberg, D. M. (1971). "Preparation and properties of...
    3 KB (192 words) - 02:35, 30 January 2023