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Hydroxymethylglutaryl-CoA synthase

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Bearfield JC, Keeling CI, Young S, Blomquist GJ, Tittiger C (April 2006). "Isolation, endocrine regulation and mRNA distribution of the 3-hydroxy-3-methylglutaryl coenzyme A synthase (HMG-S) gene from the pine engraver, Ips pini (Coleoptera: Scolytidae)".
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Steussy CN, Vartia AA, Burgner JW, Sutherlin A, Rodwell VW, Stauffacher CV (November 2005). "X-ray crystal structures of HMG-CoA synthase from Enterococcus faecalis and a complex with its second substrate/inhibitor acetoacetyl-CoA".
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Steussy CN, Robison AD, Tetrick AM, Knight JT, Rodwell VW, Stauffacher CV, Sutherlin AL (December 2006). "A structural limitation on enzyme activity: the case of HMG-CoA synthase".
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for the mitochondrial form of the enzyme has three sterol regulatory elements in the 5' flanking region. These elements are responsible for decreased
2627: 2034: 1980: 2634: 2398: 2148: 1437: 980:); in humans the cytosolic form has only 60.6% amino acid identity with the mitochondrial form of the enzyme. HMG-CoA is also found in other 1422: 3161: 3156: 2622: 1917: 1760: 1816: 2794: 2336: 2292: 2195: 1975: 1682: 1615: 221: 72: 2284: 2220: 1574: 2827: 2578: 2516: 1801: 1620: 1430: 2573: 2568: 1610: 1055: 209: 60: 2511: 2092: 1922: 1686: 202: 53: 2526: 2438: 2073: 1602: 1051: 682:. This results in shunting of excess acetyl-CoA into the ketone synthesis pathway via HMG-CoA, leading to the development of 628: 2983: 593: 447: 2691: 2671: 2585: 2408: 2115: 2063: 1996: 3098: 2612: 2506: 2501: 1703: 2774: 2548: 2403: 1957: 1534: 1120: 1116: 1112: 1108: 1529: 2784: 2617: 2491: 1524: 2968: 1080:, isoprenoid precursors are generally synthesised via an alternative, non-mevalonate pathway, however a number of 3171: 3084: 3071: 3058: 3045: 3032: 3019: 3006: 2732: 2708: 2659: 2543: 2496: 2471: 2431: 2068: 1783: 1744: 1726: 1558: 689: 2978: 2932: 2875: 2737: 2462: 1970: 1136: 1039: 886: 732: 663: 613: 113: 581: 435: 3176: 2880: 2779: 2686: 1579: 2747: 2742: 2607: 2039: 1645: 1593: 1501: 707: 214: 65: 2901: 2820: 2727: 2722: 2001: 1461: 683: 655: 2973: 577: 431: 2789: 2676: 2663: 2563: 2173: 2054: 1511: 1220: 933: 267: 130: 2937: 2521: 2044: 1721: 1675: 1539: 1516: 1047: 901: 752: 2870: 2359: 1875: 1867: 1292: 1100: 1015: 2245: 3166: 2752: 2481: 2370: 2300: 1965: 1946: 1496: 1411: 1372: 1336: 1284: 1248: 1189: 1104: 568: 422: 800:
acetyl-CoA:acetoacetyl-CoA C-acetyltransferase (thioester-hydrolysing, carboxymethyl-forming)
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Goldstein J.L., Brown M.S. (1990) Regulation of the mevalonate pathway. Nature 343, 425-430
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utilise a mevalonate pathway involving HMG-CoA synthase that is parallel to that found in
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Theisen MJ, Misra I, Saadat D, Campobasso N, Miziorko HM, Harrison DH (November 2004).
1406: 1389: 1243: 1208: 1184: 1158:"3-hydroxy-3-methylglutaryl-CoA synthase intermediate complex observed in "real-time"" 1157: 3150: 2906: 2865: 1280: 1081: 1031: 977: 1296: 497: 351: 2855: 2140: 1465: 1027: 1004:
The cytosolic form is the starting point of the mevalonate pathway, which leads to
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3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)
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staphylococcus aureus 3-hydroxy-3-methylglutaryl-coa synthase
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staphylococcus aureus 3-hydroxy-3-methylglutaryl-coa synthase
688: 1390:"The biosynthesis of beta-hydroxy-beta-methylglutaric acid" 2805: 643:(HMG-CoA). This reaction comprises the second step in the 22:
3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)
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that convert acyl groups into alkyl groups on transfer.
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This article incorporates text from the public domain
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This article incorporates text from the public domain
808:)-3-hydroxy-3-methylglutaryl-CoA acetoacetyl-CoA-lyase 3096: 710: 2338: 2294: 2286: 2222: 2197: 462:
Hydroxymethylglutaryl-coenzyme A synthase C terminal
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Hydroxymethylglutaryl-coenzyme A synthase N terminal
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This reaction is overactivated in patients with 1103:have been solved for this class of enzymes, with 908:(its second substrate) leads to the formation of 824:3-hydroxy-3-methylglutaryl coenzyme A synthetase 670:deficiency and the exhaustion of substrates for 1026:The mitochondrial form is responsible for the 832:3-hydroxy-3-methylglutaryl-coenzyme A synthase 820:3-hydroxy-3-methylglutaryl coenzyme A synthase 2821: 2439: 1832: 1438: 1050:is high in animals: the same is observed for 1008:and other sterolic and isoprenoid compounds. 836:beta-hydroxy-beta-methylglutaryl-CoA synthase 8: 2645:2-acylglycerol-3-phosphate O-acyltransferase 2640:1-acylglycerol-3-phosphate O-acyltransferase 654:pathway. HMG-CoA is an intermediate in both 2828: 2814: 2806: 2446: 2432: 2424: 2261: 2135: 2021: 1880: 1839: 1825: 1817: 1635: 1598: 1483: 1445: 1431: 1423: 930:valine, leucine and isoleucine degradation 926:synthesis and degradation of ketone bodies 541: 467: 395: 321: 279: 142: 1405: 1242: 1232: 1183: 1173: 1135:at the U.S. National Library of Medicine 848:hydroxymethylglutaryl coenzyme A synthase 816:3-hydroxy-3-methylglutaryl CoA synthetase 766:In humans, the protein is encoded by the 709: 2035:Farnesyl-diphosphate farnesyltransferase 3103: 1981:Isopentenyl-diphosphate delta isomerase 1148: 873:in the first step of the reaction: the 862:HMG-CoA synthase contains an important 828:3-hydroxy-3-methylglutaryl-CoA synthase 2635:Glycerol-3-phosphate O-acyltransferase 2399:Steroidogenic acute regulatory protein 1046:responsible for enzyme synthesis when 728:)-3-hydroxy-3-methylglutaryl-CoA + CoA 458: 312: 175: 18: 2628:Lecithin—cholesterol acyltransferase 778:This enzyme belongs to the family of 759:)-3-hydroxy-3-methylglutaryl-CoA and 7: 2623:Glyceronephosphate O-acyltransferase 1761:Hydroxyacyl-Coenzyme A dehydrogenase 802:. Other names in common use include 666:if left untreated, due to prolonged 2795:Sulfoacetaldehyde acetyltransferase 2487:Acetyl-Coenzyme A acetyltransferase 1976:Pyrophosphomevalonate decarboxylase 1894:Acetyl-Coenzyme A acetyltransferase 1616:Carnitine-acylcarnitine translocase 844:acetoacetyl coenzyme A transacetase 717:{\displaystyle \rightleftharpoons } 2711:: converted into alkyl on transfer 1575:Glycerol-3-phosphate dehydrogenase 607:hydroxymethylglutaryl-CoA synthase 14: 2517:Chloramphenicol acetyltransferase 1802:Long-chain-aldehyde dehydrogenase 1621:Carnitine palmitoyltransferase II 3106: 2569:Carnitine O-palmitoyltransferase 1611:Carnitine palmitoyltransferase I 1281:10.1111/j.1365-2583.2006.00627.x 1056:low density lipoprotein receptor 2512:Beta-galactoside transacetylase 2149:Cholesterol side-chain cleavage 2093:24-Dehydrocholesterol reductase 1923:3-hydroxybutyrate dehydrogenase 2527:Serotonin N-acetyl transferase 2474:: other than amino-acyl groups 2074:7-Dehydrocholesterol reductase 1052:3-hydroxy-3-methylglutaryl-CoA 920:This enzyme participates in 3 889:) to produce an acetyl-enzyme 1: 2692:Keratinocyte transglutaminase 2672:Gamma-glutamyl transpeptidase 2586:Serine C-palmitoyltransferase 2409:Reverse cholesterol transport 1997:Dimethylallyltranstransferase 1407:10.1016/S0021-9258(18)70822-3 545:Available protein structures: 399:Available protein structures: 2613:Aminolevulinic acid synthase 2507:Acetyl-CoA C-acyltransferase 2502:Dihydrolipoyl transacetylase 1704:Acetyl-CoA C-acyltransferase 1213:Proc. Natl. Acad. Sci. U.S.A 1207:Bahnson BJ (November 2004). 1162:Proc. Natl. Acad. Sci. U.S.A 3162:Genes on human chromosome 1 3157:Genes on human chromosome 5 2775:2-hydroxyglutarate synthase 2404:Cholesterol total synthesis 1535:3-Hydroxyacyl ACP dehydrase 944:HMG-CoA synthase occurs in 3193: 3132: 3118: 2785:2-isopropylmalate synthase 2618:Beta-ketoacyl-ACP synthase 2492:N-Acetylglutamate synthase 2116:Cholesterol 7α-hydroxylase 2064:Lanosterol 14α-demethylase 1525:Beta-ketoacyl-ACP synthase 968:, there are two different 2984:Michaelis–Menten kinetics 2733:Decylhomocitrate synthase 2544:Histone acetyltransferase 2497:Choline acetyltransferase 2069:Sterol-C5-desaturase-like 1784:Malonyl-CoA decarboxylase 1745:Propionyl-CoA carboxylase 1727:2,4 Dienoyl-CoA reductase 1559:Stearoyl-CoA desaturase-1 693:HMG-CoA synthase reaction 540: 466: 394: 320: 278: 141: 2876:Diffusion-limited enzyme 2738:2-methylcitrate synthase 1971:Phosphomevalonate kinase 1530:Î’-Ketoacyl ACP reductase 1269:Insect Molecular Biology 1137:Medical Subject Headings 798:of this enzyme class is 664:diabetes mellitus type 1 2780:3-propylmalate synthase 2687:Tissue transglutaminase 1234:10.1073/pnas.0407418101 1175:10.1073/pnas.0405809101 869:residue that acts as a 2748:3-ethylmalate synthase 2743:2-ethylmalate synthase 2608:Acyltransferase like 2 2040:Squalene monooxygenase 1646:Acyl CoA dehydrogenase 1502:Acetyl-CoA carboxylase 1068: 1018: 770:gene on chromosome 5. 718: 694: 2969:Eadie–Hofstee diagram 2902:Allosteric regulation 2728:Citrate (Re)-synthase 2723:Decylcitrate synthase 2664:Aminoacyltransferases 2564:palmitoyltransferases 2121:Sterol 27-hydroxylase 2002:Geranyl pyrophosphate 1462:fatty acid metabolism 1064: 1014: 782:, specifically those 719: 692: 684:diabetic ketoacidosis 656:cholesterol synthesis 2979:Lineweaver–Burk plot 2790:Homocitrate synthase 2677:Peptidyl transferase 2055:7-Dehydrocholesterol 1512:Fatty acid synthesis 940:Species distribution 934:butanoate metabolism 708: 704:O + acetoacetyl-CoA 2522:N-acetyltransferase 2045:Lanosterol synthase 1722:Enoyl CoA isomerase 1676:Enoyl-CoA hydratase 1540:Enoyl ACP reductase 1517:Fatty acid synthase 1225:2004PNAS..10116399B 1099:As of late 2007, 4 1048:dietary cholesterol 902:nucleophilic attack 735:of this enzyme are 2938:Enzyme superfamily 2871:Enzyme promiscuity 2482:acetyltransferases 2360:Steroid metabolism 1876:Mevalonate pathway 1868:steroid metabolism 1095:Structural studies 1069: 1019: 1016:Mevalonate pathway 922:metabolic pathways 751:, whereas its two 714: 695: 3094: 3093: 2803: 2802: 2753:ATP citrate lyase 2421: 2420: 2417: 2416: 2371:Steroid sulfatase 2348: 2347: 2101: 2100: 2010: 2009: 1966:Mevalonate kinase 1947:HMG-CoA reductase 1814: 1813: 1810: 1809: 1769: 1768: 1588: 1587: 1497:ATP citrate lyase 1388:RUDNEY H (1957). 1369:10.1021/bi051487x 1333:10.1021/bi061505q 1219:(47): 16399–400. 900:. The subsequent 812:(CoA-acetylating) 605:In biochemistry, 603: 602: 599: 598: 594:structure summary 457: 456: 453: 452: 448:structure summary 311: 310: 307: 306: 174: 173: 170: 169: 3184: 3172:Protein families 3111: 3110: 3102: 2974:Hanes–Woolf plot 2917:Enzyme activator 2912:Enzyme inhibitor 2886:Enzyme catalysis 2830: 2823: 2816: 2807: 2763:HMG-CoA synthase 2718:Citrate synthase 2682:Transglutaminase 2459:acyltransferases 2448: 2441: 2434: 2425: 2379:sulfotransferase 2340: 2296: 2288: 2262: 2224: 2199: 2136: 2022: 1901:(regulated step) 1899:HMG-CoA synthase 1881: 1852:lipid metabolism 1841: 1834: 1827: 1818: 1636: 1599: 1568:Triacyl glycerol 1484: 1458:lipid metabolism 1447: 1440: 1433: 1424: 1419: 1409: 1381: 1380: 1363:(43): 14256–67. 1351: 1345: 1344: 1327:(48): 14407–14. 1316: 1310: 1307: 1301: 1300: 1263: 1257: 1256: 1246: 1236: 1204: 1198: 1197: 1187: 1177: 1153: 1133:HMG-CoA+synthase 1107:accession codes 893:, releasing the 840:HMG-CoA synthase 784:acyltransferases 723: 721: 720: 715: 611:HMG-CoA synthase 542: 471: 459: 396: 325: 313: 280: 176: 143: 19: 16:Class of enzymes 3192: 3191: 3187: 3186: 3185: 3183: 3182: 3181: 3147: 3146: 3145: 3131: 3117: 3105: 3097: 3095: 3090: 3002:Oxidoreductases 2988: 2964:Enzyme kinetics 2952: 2948:List of enzymes 2921: 2890: 2861:Catalytic triad 2839: 2834: 2804: 2799: 2758:Malate synthase 2703: 2654: 2466: 2452: 2422: 2413: 2353:Other/ungrouped 2344: 2317: 2275:17α-Hydroxylase 2250: 2246:11β-Hydroxylase 2191:17α-Hydroxylase 2160:corticosteroids 2153: 2131:Steroidogenesis 2125: 2097: 2078: 2049: 2006: 1985: 1951: 1932: 1904: 1870: 1845: 1815: 1806: 1788: 1765: 1749: 1731: 1708: 1625: 1584: 1563: 1544: 1515: 1506: 1475: 1451: 1387: 1384: 1353: 1352: 1348: 1318: 1317: 1313: 1308: 1304: 1265: 1264: 1260: 1206: 1205: 1201: 1168:(47): 16442–7. 1155: 1154: 1150: 1146: 1129: 1097: 1074: 1024: 1002: 972:of the enzyme ( 962: 942: 918: 916:Biological role 906:acetoacetyl-CoA 860: 796:systematic name 792: 776: 749:acetoacetyl-CoA 744: 729: 706: 705: 703: 697: 672:gluconeogenesis 625:acetoacetyl-CoA 623:condenses with 474: 328: 17: 12: 11: 5: 3190: 3188: 3180: 3179: 3177:Human proteins 3174: 3169: 3164: 3159: 3149: 3148: 3116: 3115: 3092: 3091: 3089: 3088: 3075: 3062: 3049: 3036: 3023: 3010: 2996: 2994: 2990: 2989: 2987: 2986: 2981: 2976: 2971: 2966: 2960: 2958: 2954: 2953: 2951: 2950: 2945: 2940: 2935: 2929: 2927: 2926:Classification 2923: 2922: 2920: 2919: 2914: 2909: 2904: 2898: 2896: 2892: 2891: 2889: 2888: 2883: 2878: 2873: 2868: 2863: 2858: 2853: 2847: 2845: 2841: 2840: 2835: 2833: 2832: 2825: 2818: 2810: 2801: 2800: 2798: 2797: 2792: 2787: 2782: 2777: 2772: 2771: 2770: 2760: 2755: 2750: 2745: 2740: 2735: 2730: 2725: 2720: 2714: 2712: 2705: 2704: 2702: 2701: 2700: 2699: 2694: 2689: 2679: 2674: 2668: 2666: 2656: 2655: 2653: 2652: 2647: 2642: 2637: 2631: 2630: 2625: 2620: 2615: 2610: 2601: 2600: 2599: 2598: 2593: 2583: 2582: 2581: 2576: 2559: 2558: 2557: 2556: 2551: 2541: 2540: 2539: 2534: 2529: 2519: 2514: 2509: 2504: 2499: 2494: 2489: 2477: 2475: 2468: 2467: 2453: 2451: 2450: 2443: 2436: 2428: 2419: 2418: 2415: 2414: 2412: 2411: 2406: 2401: 2395: 2394: 2393: 2392: 2387: 2375: 2374: 2373: 2356: 2354: 2350: 2349: 2346: 2345: 2343: 2342: 2334: 2328: 2326: 2319: 2318: 2316: 2315: 2314: 2313: 2308: 2298: 2290: 2282: 2277: 2271: 2269: 2259: 2252: 2251: 2249: 2248: 2243: 2241:21-Hydroxylase 2238: 2237: 2236: 2231: 2214: 2213: 2212: 2211: 2206: 2193: 2177: 2176: 2174:18-Hydroxylase 2164: 2162: 2155: 2154: 2152: 2151: 2145: 2143: 2133: 2127: 2126: 2124: 2123: 2118: 2112: 2110: 2103: 2102: 2099: 2098: 2096: 2095: 2089: 2087: 2080: 2079: 2077: 2076: 2071: 2066: 2060: 2058: 2051: 2050: 2048: 2047: 2042: 2037: 2031: 2029: 2019: 2012: 2011: 2008: 2007: 2005: 2004: 1999: 1993: 1991: 1987: 1986: 1984: 1983: 1978: 1973: 1968: 1962: 1960: 1953: 1952: 1950: 1949: 1943: 1941: 1939:Mevalonic acid 1934: 1933: 1931: 1930: 1925: 1920: 1914: 1912: 1906: 1905: 1903: 1902: 1896: 1890: 1888: 1878: 1872: 1871: 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597: 596: 591: 585: 584: 571: 565: 564: 554: 547: 546: 538: 537: 532: 526: 525: 520: 514: 513: 508: 501: 500: 495: 489: 488: 487:HMG_CoA_synt_C 485: 481: 480: 476: 475: 472: 464: 463: 455: 454: 451: 450: 445: 439: 438: 425: 419: 418: 408: 401: 400: 392: 391: 386: 380: 379: 374: 368: 367: 362: 355: 354: 349: 343: 342: 341:HMG_CoA_synt_N 339: 335: 334: 330: 329: 326: 318: 317: 309: 308: 305: 304: 299: 295: 294: 289: 285: 284: 276: 275: 265: 259: 258: 254: 253: 248: 242: 241: 236: 230: 229: 224: 218: 217: 212: 206: 205: 200: 194: 193: 190: 186: 185: 181: 180: 172: 171: 168: 167: 162: 158: 157: 152: 148: 147: 139: 138: 128: 122: 121: 116: 110: 109: 105: 104: 99: 93: 92: 87: 81: 80: 75: 69: 68: 63: 57: 56: 51: 45: 44: 41: 37: 36: 33: 29: 28: 24: 23: 15: 13: 10: 9: 6: 4: 3: 2: 3189: 3178: 3175: 3173: 3170: 3168: 3165: 3163: 3160: 3158: 3155: 3154: 3152: 3144: 3140: 3136: 3130: 3126: 3122: 3114: 3109: 3104: 3100: 3086: 3082: 3081: 3076: 3073: 3069: 3068: 3063: 3060: 3056: 3055: 3050: 3047: 3043: 3042: 3037: 3034: 3030: 3029: 3024: 3021: 3017: 3016: 3011: 3008: 3004: 3003: 2998: 2997: 2995: 2991: 2985: 2982: 2980: 2977: 2975: 2972: 2970: 2967: 2965: 2962: 2961: 2959: 2955: 2949: 2946: 2944: 2943:Enzyme family 2941: 2939: 2936: 2934: 2931: 2930: 2928: 2924: 2918: 2915: 2913: 2910: 2908: 2907:Cooperativity 2905: 2903: 2900: 2899: 2897: 2893: 2887: 2884: 2882: 2879: 2877: 2874: 2872: 2869: 2867: 2866:Oxyanion hole 2864: 2862: 2859: 2857: 2854: 2852: 2849: 2848: 2846: 2842: 2838: 2831: 2826: 2824: 2819: 2817: 2812: 2811: 2808: 2796: 2793: 2791: 2788: 2786: 2783: 2781: 2778: 2776: 2773: 2769: 2766: 2765: 2764: 2761: 2759: 2756: 2754: 2751: 2749: 2746: 2744: 2741: 2739: 2736: 2734: 2731: 2729: 2726: 2724: 2721: 2719: 2716: 2715: 2713: 2710: 2706: 2698: 2695: 2693: 2690: 2688: 2685: 2684: 2683: 2680: 2678: 2675: 2673: 2670: 2669: 2667: 2665: 2661: 2657: 2651: 2648: 2646: 2643: 2641: 2638: 2636: 2633: 2632: 2629: 2626: 2624: 2621: 2619: 2616: 2614: 2611: 2609: 2606: 2603: 2602: 2597: 2594: 2592: 2589: 2588: 2587: 2584: 2580: 2577: 2575: 2572: 2571: 2570: 2567: 2565: 2561: 2560: 2555: 2552: 2550: 2547: 2546: 2545: 2542: 2538: 2535: 2533: 2530: 2528: 2525: 2524: 2523: 2520: 2518: 2515: 2513: 2510: 2508: 2505: 2503: 2500: 2498: 2495: 2493: 2490: 2488: 2485: 2483: 2479: 2478: 2476: 2473: 2469: 2464: 2460: 2456: 2449: 2444: 2442: 2437: 2435: 2430: 2429: 2426: 2410: 2407: 2405: 2402: 2400: 2397: 2396: 2391: 2388: 2386: 2383: 2382: 2381: 2380: 2376: 2372: 2369: 2368: 2367: 2366: 2361: 2358: 2357: 2355: 2351: 2341: 2335: 2333: 2330: 2329: 2327: 2325: 2320: 2312: 2309: 2307: 2304: 2303: 2302: 2299: 2297: 2291: 2289: 2283: 2281: 2278: 2276: 2273: 2272: 2270: 2268: 2263: 2260: 2258: 2253: 2247: 2244: 2242: 2239: 2235: 2232: 2230: 2227: 2226: 2225: 2219: 2216: 2215: 2210: 2207: 2205: 2202: 2201: 2200: 2194: 2192: 2189: 2187: 2183: 2179: 2178: 2175: 2172: 2170: 2166: 2165: 2163: 2161: 2156: 2150: 2147: 2146: 2144: 2142: 2137: 2134: 2132: 2128: 2122: 2119: 2117: 2114: 2113: 2111: 2109: 2104: 2094: 2091: 2090: 2088: 2085: 2081: 2075: 2072: 2070: 2067: 2065: 2062: 2061: 2059: 2056: 2052: 2046: 2043: 2041: 2038: 2036: 2033: 2032: 2030: 2028: 2023: 2020: 2018: 2013: 2003: 2000: 1998: 1995: 1994: 1992: 1988: 1982: 1979: 1977: 1974: 1972: 1969: 1967: 1964: 1963: 1961: 1959: 1954: 1948: 1945: 1944: 1942: 1940: 1935: 1929: 1926: 1924: 1921: 1919: 1918:HMG-CoA lyase 1916: 1915: 1913: 1911: 1907: 1900: 1897: 1895: 1892: 1891: 1889: 1887: 1882: 1879: 1877: 1873: 1869: 1865: 1861: 1857: 1853: 1849: 1842: 1837: 1835: 1830: 1828: 1823: 1822: 1819: 1803: 1800: 1799: 1797: 1795: 1791: 1785: 1782: 1781: 1779: 1777: 1772: 1762: 1759: 1758: 1756: 1752: 1746: 1743: 1742: 1740: 1738: 1734: 1728: 1725: 1723: 1720: 1719: 1717: 1715: 1711: 1705: 1702: 1701: 1698: 1695: 1693: 1690: 1688: 1684: 1681: 1680: 1677: 1674: 1671: 1668: 1666: 1663: 1661: 1658: 1656: 1653: 1651: 1647: 1644: 1643: 1641: 1637: 1634: 1632: 1628: 1622: 1619: 1617: 1614: 1612: 1609: 1608: 1606: 1604: 1600: 1597: 1595: 1591: 1581: 1578: 1576: 1573: 1572: 1570: 1566: 1560: 1557: 1556: 1554: 1552: 1547: 1541: 1538: 1536: 1533: 1531: 1528: 1526: 1523: 1522: 1520: 1518: 1513: 1509: 1503: 1500: 1498: 1495: 1494: 1492: 1489: 1485: 1482: 1478: 1474: 1471: 1467: 1463: 1459: 1455: 1448: 1443: 1441: 1436: 1434: 1429: 1428: 1425: 1417: 1413: 1408: 1403: 1400:(1): 363–77. 1399: 1395: 1394:J. Biol. Chem 1391: 1386: 1385: 1378: 1374: 1370: 1366: 1362: 1358: 1350: 1347: 1342: 1338: 1334: 1330: 1326: 1322: 1315: 1312: 1306: 1303: 1298: 1294: 1290: 1286: 1282: 1278: 1275:(2): 187–95. 1274: 1270: 1262: 1259: 1254: 1250: 1245: 1240: 1235: 1230: 1226: 1222: 1218: 1214: 1210: 1203: 1200: 1195: 1191: 1186: 1181: 1176: 1171: 1167: 1163: 1159: 1152: 1149: 1143: 1138: 1134: 1131: 1130: 1126: 1124: 1122: 1118: 1114: 1110: 1106: 1102: 1094: 1092: 1090: 1086: 1083: 1082:Gram-positive 1079: 1071: 1067: 1063: 1059: 1057: 1053: 1049: 1045: 1041: 1040:transcription 1037: 1033: 1032:ketone bodies 1029: 1022:Mitochondrial 1021: 1017: 1013: 1009: 1007: 999: 997: 995: 991: 987: 983: 979: 978:mitochondrial 975: 971: 967: 959: 957: 955: 951: 947: 939: 937: 935: 931: 927: 923: 915: 913: 911: 907: 903: 899: 896: 892: 888: 884: 880: 876: 872: 868: 865: 857: 855: 853: 849: 845: 841: 837: 833: 829: 825: 821: 817: 813: 809: 805: 801: 797: 789: 787: 785: 781: 773: 771: 769: 764: 762: 758: 754: 750: 746: 738: 734: 727: 698: 691: 687: 685: 681: 677: 673: 669: 665: 661: 657: 653: 650: 646: 642: 640: 636: 632: 626: 622: 618: 615: 612: 608: 595: 592: 590: 586: 583: 579: 575: 572: 570: 566: 562: 558: 555: 552: 548: 543: 539: 536: 533: 531: 527: 524: 521: 519: 515: 512: 509: 506: 502: 499: 496: 494: 490: 486: 482: 477: 470: 465: 460: 449: 446: 444: 440: 437: 433: 429: 426: 424: 420: 416: 412: 409: 406: 402: 397: 393: 390: 387: 385: 381: 378: 375: 373: 369: 366: 363: 360: 356: 353: 350: 348: 344: 340: 336: 331: 324: 319: 314: 303: 300: 296: 293: 290: 286: 281: 277: 274: 273: 269: 266: 264: 260: 255: 252: 249: 247: 243: 240: 237: 235: 231: 228: 225: 223: 219: 216: 213: 211: 207: 204: 201: 199: 195: 191: 187: 182: 177: 166: 163: 159: 156: 153: 149: 144: 140: 137: 136: 132: 129: 127: 123: 120: 117: 115: 111: 106: 103: 100: 98: 94: 91: 88: 86: 82: 79: 76: 74: 70: 67: 64: 62: 58: 55: 52: 50: 46: 42: 38: 34: 30: 25: 20: 3080:Translocases 3077: 3064: 3051: 3038: 3025: 3015:Transferases 3012: 2999: 2856:Binding site 2762: 2604: 2562: 2480: 2455:Transferases 2377: 2363: 2301:5α-Reductase 2257:sex hormones 2217: 2180: 2167: 2141:pregnenolone 1466:triglyceride 1397: 1393: 1360: 1357:Biochemistry 1356: 1349: 1324: 1321:Biochemistry 1320: 1314: 1305: 1272: 1268: 1261: 1216: 1212: 1202: 1165: 1161: 1151: 1098: 1075: 1028:biosynthesis 1025: 1003: 963: 943: 919: 861: 851: 847: 843: 839: 835: 831: 827: 823: 819: 815: 811: 807: 803: 799: 793: 790:Nomenclature 780:transferases 777: 767: 765: 756: 730: 725: 696: 680:oxaloacetate 652:biosynthesis 638: 634: 630: 610: 606: 604: 270: 133: 40:Alt. symbols 2851:Active site 2697:Factor XIII 2280:17,20-Lyase 2169:aldosterone 2084:Desmosterol 2017:cholesterol 1928:Thiophorase 1910:Ketogenesis 1860:cholesterol 1714:Unsaturated 1594:Degradation 1551:desaturases 1549:Fatty acid 1488:Malonyl-CoA 1066:Ketogenesis 1006:cholesterol 966:vertebrates 885:(its first 875:acetylation 871:nucleophile 660:ketogenesis 647:-dependent 641:lutaryl-CoA 479:Identifiers 333:Identifiers 292:Swiss-model 184:Identifiers 155:Swiss-model 27:Identifiers 3151:Categories 3054:Isomerases 3028:Hydrolases 2895:Regulation 2108:Bile acids 2027:lanosterol 1862:synthesis 1848:Metabolism 1776:acetyl-CoA 1580:Thiokinase 1470:fatty acid 1454:Metabolism 1144:References 1101:structures 1089:eukaryotes 982:eukaryotes 960:Eukaryotes 946:eukaryotes 898:coenzyme A 883:acetyl-CoA 737:acetyl-CoA 733:substrates 678:, notably 649:isoprenoid 645:mevalonate 621:acetyl-CoA 557:structures 411:structures 288:Structures 283:Search for 257:Other data 151:Structures 146:Search for 108:Other data 3143:IPR013528 3129:IPR013746 2933:EC number 2365:sulfatase 2332:Aromatase 2324:estrogens 2267:androgens 2186:cortisone 1794:Aldehydes 1737:Odd chain 1490:synthesis 1480:Synthesis 1085:pathogens 1000:Cytosolic 974:cytosolic 891:thioester 887:substrate 864:catalytic 858:Mechanism 712:⇌ 676:TCA cycle 633:ydroxy-3- 535:PDOC00942 523:IPR013746 389:PDOC00942 377:IPR013528 239:NM_005518 198:NCBI gene 114:EC number 90:NM_002130 49:NCBI gene 3167:EC 2.3.3 3139:InterPro 3125:InterPro 2957:Kinetics 2881:Cofactor 2844:Activity 2549:P300/CBP 2182:cortisol 1990:Geranyl- 1416:13449080 1377:16245942 1341:17128980 1297:46317830 1289:16640729 1253:15546978 1194:15498869 1078:bacteria 1072:Bacteria 1054:and the 984:such as 970:isozymes 954:bacteria 867:cysteine 753:products 674:and the 627:to form 617:2.3.3.10 574:RCSB PDB 518:InterPro 428:RCSB PDB 372:InterPro 302:InterPro 165:InterPro 119:2.3.3.10 3113:Biology 3067:Ligases 2837:Enzymes 2390:SULT2A1 2385:SULT1A1 1886:HMG-CoA 1864:enzymes 1856:ketones 1639:General 1473:enzymes 1221:Bibcode 1044:message 1042:of the 986:insects 950:archaea 910:HMG-CoA 895:reduced 877:of the 668:insulin 530:PROSITE 498:PF08540 384:PROSITE 352:PF01154 298:Domains 272:p13-p12 246:UniProt 161:Domains 135:p14-p13 97:UniProt 3099:Portal 3041:Lyases 2768:HMGCS2 2605:other: 2596:SPTLC2 2591:SPTLC1 2532:HGSNAT 1670:ACADSB 1665:ACADVL 1414:  1375:  1339:  1295:  1287:  1251:  1244:534547 1241:  1192:  1185:534525 1182:  1139:(MeSH) 1119:, and 1034:. The 992:, and 990:plants 932:, and 879:enzyme 850:, and 768:HMGCS1 747:, and 731:The 3 589:PDBsum 563:  553:  511:CL0046 484:Symbol 443:PDBsum 417:  407:  365:CL0046 338:Symbol 268:Chr. 1 251:P54868 234:RefSeq 227:600234 192:HMGCS2 189:Symbol 131:Chr. 5 102:Q01581 85:RefSeq 78:142940 35:HMGCS1 32:Symbol 2993:Types 2709:2.3.3 2660:2.3.2 2650:ABHD5 2537:ARD1A 2472:2.3.1 2218:both: 1958:DMAPP 1754:Other 1697:HADHB 1692:HADHA 1660:ACADS 1655:ACADM 1650:ACADL 1293:S2CID 994:fungi 755:are ( 637:ethyl 263:Locus 126:Locus 43:HMGCS 3137:and 3135:Pfam 3123:and 3121:Pfam 3085:list 3078:EC7 3072:list 3065:EC6 3059:list 3052:EC5 3046:list 3039:EC4 3033:list 3026:EC3 3020:list 3013:EC2 3007:list 3000:EC1 2579:CPT2 2574:CPT1 2554:NAT2 2465:2.3) 2337:17β- 2293:17β- 2196:11β- 2086:path 2057:path 1687:HADH 1468:and 1412:PMID 1373:PMID 1337:PMID 1285:PMID 1249:PMID 1190:PMID 1121:2P8U 1117:1XPM 1113:1XPL 1109:1XPK 1036:gene 976:and 794:The 658:and 582:PDBj 578:PDBe 561:ECOD 551:Pfam 507:clan 505:Pfam 493:Pfam 436:PDBj 432:PDBe 415:ECOD 405:Pfam 361:clan 359:Pfam 347:Pfam 222:OMIM 215:5008 210:HGNC 203:3158 73:OMIM 66:5007 61:HGNC 54:3157 2339:HSD 2322:To 2295:HSD 2287:HSD 2285:3β- 2265:To 2255:To 2223:HSD 2221:3β- 2198:HSD 2158:To 2139:To 2106:To 2025:To 2015:To 1956:To 1937:To 1884:To 1774:To 1683:MTP 1402:doi 1398:227 1365:doi 1329:doi 1277:doi 1239:PMC 1229:doi 1217:101 1180:PMC 1170:doi 1166:101 1105:PDB 1076:In 1030:of 964:In 904:on 881:by 761:CoA 609:or 569:PDB 423:PDB 3153:: 3141:: 3127:: 2662:: 2463:EC 2457:: 2362:: 1854:– 1850:: 1685:: 1464:, 1460:/ 1456:: 1410:. 1396:. 1392:. 1371:. 1361:44 1359:. 1335:. 1325:45 1323:. 1291:. 1283:. 1273:15 1271:. 1247:. 1237:. 1227:. 1215:. 1211:. 1188:. 1178:. 1164:. 1160:. 1123:. 1115:, 1111:, 1091:. 1058:. 996:. 988:, 956:. 948:, 936:. 928:, 924:: 912:. 854:. 846:, 842:, 838:, 834:, 830:, 826:, 822:, 818:, 814:, 810:, 763:. 739:, 686:. 629:3- 614:EC 580:; 576:; 559:/ 434:; 430:; 413:/ 3101:: 3087:) 3083:( 3074:) 3070:( 3061:) 3057:( 3048:) 3044:( 3035:) 3031:( 3022:) 3018:( 3009:) 3005:( 2829:e 2822:t 2815:v 2566:: 2484:: 2461:( 2447:e 2440:t 2433:v 2311:2 2306:1 2234:2 2229:1 2209:2 2204:1 2188:: 2184:/ 2171:: 1866:/ 1858:/ 1840:e 1833:t 1826:v 1672:) 1648:( 1514:/ 1446:e 1439:t 1432:v 1418:. 1404:: 1379:. 1367:: 1343:. 1331:: 1299:. 1279:: 1255:. 1231:: 1223:: 1196:. 1172:: 806:S 804:( 757:S 745:O 743:2 741:H 726:S 724:( 702:2 639:g 635:m 631:h

Index

NCBI gene
3157
HGNC
5007
OMIM
142940
RefSeq
NM_002130
UniProt
Q01581
EC number
2.3.3.10
Locus
Chr. 5
p14-p13
Swiss-model
InterPro
NCBI gene
3158
HGNC
5008
OMIM
600234
RefSeq
NM_005518
UniProt
P54868
Locus
Chr. 1
p13-p12

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