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Methylglutaconyl-CoA hydratase

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pathways. In the first, HMB is produced from by the cytosolic enzyme KIC dioxygenase (Sabourin and Bieber 1983). The cytosolic dioxygenase has been characterized extensively and differs from the mitochondrial form in that the dioxygenase enzyme is a cytosolic enzyme, whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this route of HMB formation is direct and completely dependent of liver KIC dioxygenase. Following this pathway, HMB in the cytosol is first converted to cytosolic β-hydroxy-β-methylglutaryl-CoA (HMG-CoA), which can then be directed for cholesterol synthesis (Rudney 1957) (Fig. 1). In fact, numerous biochemical studies have shown that HMB is a precursor of cholesterol (Zabin and Bloch 1951; Nissen et al. 2000).
1281: 316: 293: 215: 190: 1690: 1772:) and are present in certain patients who have the disorder. Deletions of exons 1–3 in the gene suggest that these exons are responsible for the biochemical and clinical characteristics of 3-Methylglutaconic Acuduria Type 1. These mutations cause for the deficiency of 3-methylglutaconyl-CoA hydratase which leads to the amalgamation of 3-methylglutaconyl-CoA, 3-methylglutaric acid, and 3-hydroxyisovaleric acid which eventually leads to 3-Methylglutaconic Acuduria Type 1. 322: 221: 3758: 2222:
HMB is a metabolite of the amino acid leucine (Van Koverin and Nissen 1992), an essential amino acid. The first step in HMB metabolism is the reversible transamination of leucine to that occurs mainly extrahepatically (Block and Buse 1990). Following this enzymatic reaction, may follow one of two
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Energy fuel: Eventually, most Leu is broken down, providing about 6.0kcal/g. About 60% of ingested Leu is oxidized within a few hours ... Ketogenesis: A significant proportion (40% of an ingested dose) is converted into acetyl-CoA and thereby contributes to the synthesis of ketones, steroids,
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activity, AUH also has a hydrase active-site pocket created by H2A-H3 alpha-helices and the H4A 310 helix of one subunit, and the H8 and H9 alpha-helices of the adjacent subunit within the same trimer. This active-site pocket is not affected by the change in oligomeric state when AUH is in the
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pathway. Localized in the mitochondria, AUH is responsible for the fifth step in the leucine degradation pathway and deficiencies in this enzyme's activity leads to a metabolic block in which 3-methylglutaconyl-CoA, accumulates in the
329: 228: 1259:. These functions allow AUH to show us that there could be a potential connection between mitochondrial metabolism and gene regulation. Also, reduced or overexprsessed levels of AUH can lead to defects in 2130:
Wilson JM, Fitschen PJ, Campbell B, Wilson GJ, Zanchi N, Taylor L, Wilborn C, Kalman DS, Stout JR, Hoffman JR, Ziegenfuss TN, Lopez HL, Kreider RB, Smith-Ryan AE, Antonio J (February 2013).
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Mercimek-Mahmutoglu S, Tucker T, Casey B (Nov 2011). "Phenotypic heterogeneity in two siblings with 3-methylglutaconic aciduria type I caused by a novel intragenic deletion".
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Kurimoto K, Kuwasako K, Sandercock AM, Unzai S, Robinson CV, Muto Y, Yokoyama S (May 2009). "AU-rich RNA-binding induces changes in the quaternary structure of AUH".
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in the enzyme's activity leads to increases in 3-methylglutaric acid and 3-hydroxyisovaleric acid. Another function of AUH is that it binds to an
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state of AUH depends on whether or not RNA is present. If RNA is near, the AUH will take on an asymmetric shape that loses the 3- and 2-fold
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residues were shown to be the main reason why AUH is able to bind to RNA rather than its counterparts. Moreover, it has been found that the
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Zanchi NE, Gerlinger-Romero F, GuimarĂŁes-Ferreira L, de Siqueira Filho MA, Felitti V, Lira FS, Seelaender M, Lancha AH (April 2011).
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rotation axes, because of realignment of the internal 3-fold axes of the trimers. Because this enzyme has weak, short-chain
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cells. AUH has a similar fold that is found in other members of the enoyl-CoA hydratase/isomerase family; however, it is a
122:, AU RNA binding protein/enoyl-CoA hydratase, Methylglutaconyl-CoA hydratase, AU RNA binding methylglutaconyl-CoA hydratase 58: 2272: 3150: 2747: 2470: 1808: 1699: 1436: 1405: 1385: 2950: 2440: 315: 3477: 3248: 2945: 2862: 2771: 2331: 292: 2475: 3439: 2617: 1833: 1812: 1611: 3009: 2485: 1744:
The lack of AUH is most impactful to the human body by causing 3-Methylglutaconic Acuduria Type 1, which is an
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Richman TR, Davies SM, Shearwood AM, Ermer JA, Scott LH, Hibbs ME, Rackham O, Filipovska A (May 2014).
17: 1936:"Crystal structure of human AUH protein, a single-stranded RNA binding homolog of enoyl-CoA hydratase" 328: 227: 3734: 3721: 3708: 3695: 3682: 3669: 3656: 3618: 3145: 3140: 3093: 3054: 2699: 1363: 1351: 321: 220: 3628: 3582: 3525: 3357: 3276: 3103: 2680: 2302: 2296: 1643: 1236: 1073: 960: 956: 952: 944: 897: 893: 889: 881: 139: 118: 3022: 3004: 2132:"International Society of Sports Nutrition Position Stand: beta-hydroxy-beta-methylbutyrate (HMB)" 3530: 2834: 2809: 2737: 2719: 2500:– this diagram does not include the pathway for β-leucine synthesis via leucine 2,3-aminomutase) 2480: 2186:"HMB supplementation: clinical and athletic performance-related effects and mechanisms of action" 2026:
Mack M, Schniegler-Mattox U, Peters V, Hoffmann GF, Liesert M, Buckel W, Zschocke J (May 2006).
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of leucine degradation and can range in severity from developmental delay to slowly progressive
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is mostly metabolized into isovaleryl-CoA, while only about 5% is metabolized into HMB.
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of this gene have been found to cause a disease called 3-Methylglutaconic Acuduria Type 1.
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AUUUA. AREs have been found in the 3’-untranslated regions of mRNA and they promote mRNA
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National Center for Biotechnology Information, U.S. National Library of Medicine
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National Center for Biotechnology Information, U.S. National Library of Medicine
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superfamily, but it is the only member of that family that is able to bind to
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stability. AUH is also responsible for regulating mitochondrial
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Kurimoto K, Fukai S, Nureki O, Muto Y, Yokoyama S (Dec 2001).
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Overview of all the structural information available in the
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Journal of the International Society of Sports Nutrition
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Nutrient Metabolism: Structures, Functions, and Genes
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Branched-chain alpha-keto acid dehydrogenase complex
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Branched-chain alpha-keto acid dehydrogenase complex
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Branched-chain alpha-keto acid dehydrogenase complex
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Academic Press. pp. 385–388. 2228: 1825: 1823: 1821: 1804: 1802: 1800: 2588: 338: 237: 3770: 2541:Aminocarboxymuconate-semialdehyde decarboxylase 2073: 2071: 1818: 1797: 27:Protein-coding gene in the species Homo sapiens 2021: 2019: 1975: 1973: 1971: 1894: 1892: 1830:GRCm38: Ensembl release 89: ENSMUSG00000021460 3471: 3256: 2347: 2068: 1756:gene has been seen in 10 different sites (5 2985:3-hydroxy-2-methylbutyryl-CoA dehydrogenase 2016: 1968: 1927: 1889: 1809:GRCh38: Ensembl release 89: ENSG00000148090 667:branched-chain amino acid catabolic process 3478: 3464: 3263: 3249: 2975:Branched-chain amino acid aminotransferase 2931:Branched-chain amino acid aminotransferase 2446:Branched-chain amino acid aminotransferase 2354: 2340: 1102:enzymatic activity, making it a dual-role 2956:Methylmalonate semialdehyde dehydrogenase 2295:at the U.S. National Library of Medicine 2237: 2157: 2147: 2103: 2043: 1951: 1203:to 3-hydroxy-3-methylglutaryl CoA in the 2546:Aminomuconate-semialdehyde dehydrogenase 1739: 1223:(ARE), containing clusters of the penta- 2777:1-Pyrroline-5-carboxylate dehydrogenase 1243:and is essential for mitochondrial RNA 600:methylglutaconyl-CoA hydratase activity 14: 3771: 3037:Betaine-homocysteine methyltransferase 1662: 1655: 1648: 1641: 1634: 1627: 1609: 1600: 1580: 1571: 1562: 1555: 1540: 1529: 1519: 1508: 1498: 1488: 1478: 1468: 1459: 1450: 1441: 1434: 1396: 1369: 3459: 3244: 2498:Template:Leucine metabolism in humans 2335: 1875: 1676: 1669: 1618: 1591: 1403: 1383: 1357: 1338: 1331: 1324: 1317: 1310: 343: 304: 299: 242: 201: 196: 2638:Guanidinoacetate N-methyltransferase 1780:AUH has been seen to interact with: 1420: 1345: 1301: 1287: 3151:4-Hydroxyphenylpyruvate dioxygenase 2748:Formiminotransferase cyclodeaminase 2471:Isovaleryl coenzyme A dehydrogenase 24: 2951:3-hydroxyisobutyrate dehydrogenase 2441:3-Hydroxybutyryl-CoA dehydrogenase 25: 3795: 2946:3-hydroxyisobutyryl-CoA hydrolase 2863:Gamma-glutamylcysteine synthetase 2772:Pyrroline-5-carboxylate reductase 2286: 1901:Molecular Genetics and Metabolism 437:lateral nuclear group of thalamus 3756: 2268:fatty acids, and other compounds 2045:10.1111/j.1742-4658.2006.05218.x 1688: 1279: 1126:and the AUH gene consists of 18 1058:3-Methylglutaconyl-CoA hydratase 327: 320: 314: 291: 226: 219: 213: 188: 3440:Carboxymethyloxysuccinate lyase 2618:Serine hydroxymethyltransferase 1775: 630:itaconyl-CoA hydratase activity 3343:Methylglutaconyl-CoA hydratase 3032:Homocysteine methyltransferase 3010:Methionine adenosyltransferase 2491:Methylglutaconyl-CoA hydratase 2486:Methylcrotonyl-CoA carboxylase 2293:methylglutaconyl-CoA+hydratase 1857: 1839: 1444:β-Hydroxy β-methylglutaryl-CoA 545:More reference expression data 13: 1: 3373:Uroporphyrinogen III synthase 3363:3-Isopropylmalate dehydratase 3161:Fumarylacetoacetate hydrolase 3156:Homogentisate 1,2-dioxygenase 2873:Gamma-glutamyl transpeptidase 2536:3-hydroxyanthranilate oxidase 2476:α-Ketoisocaproate dioxygenase 1953:10.1016/s0969-2126(01)00686-4 1791: 1770:single nucleotide duplication 1717:. Of the two major pathways, 1134:long, and is mainly found in 1092:enoyl-CoA hydratase/isomerase 312: 211: 1752:in adults. Mutations in the 1746:autosomal recessive disorder 1274:Leucine metabolism in humans 1113: 605:enoyl-CoA hydratase activity 461:myocardium of left ventricle 7: 3779:Genes on human chromosome 9 3485: 3390:: Acting on polysaccharides 3353:Cystathionine beta synthase 3338:3-Hydroxyacyl ACP dehydrase 3099:Methylmalonyl CoA epimerase 3050:Cystathionine beta synthase 2517:Indoleamine 2,3-dioxygenase 2273:Figure 8.57: Metabolism of 1913:10.1016/j.ymgme.2011.07.021 1190: 10: 3800: 3023:regeneration of methionine 3005:generation of homocysteine 2805:Ornithine aminotransferase 2521:Tryptophan 2,3-dioxygenase 2422:Glutaryl-CoA dehydrogenase 2417:Saccharopine dehydrogenase 1766:single nucleotide deletion 3642: 3634:Michaelis–Menten kinetics 3606: 3575: 3544: 3493: 3429: 3410: 3386: 3288: 3198: 3188: 3146:Tyrosine aminotransferase 3141:Phenylalanine hydroxylase 3126: 3116: 3094:Propionyl-CoA carboxylase 3082: 3063: 3055:Cystathionine gamma-lyase 2993: 2964: 2920: 2905: 2843: 2823: 2794: 2756: 2727: 2712: 2700:L-threonine dehydrogenase 2689: 2670: 2651: 2603: 2579: 2554: 2506: 2430: 2406: 2393: 2381: 2202:10.1007/s00726-010-0678-0 1865:"Mouse PubMed Reference:" 1847:"Human PubMed Reference:" 1372:Branched-chain α-ketoacid 1261:mitochondrial translation 1044: 1039: 1035: 1028: 1012: 993: 978: 974: 941: 937: 930: 915: 911: 878: 874: 867: 854: 850: 835: 831: 822: 809: 805: 790: 786: 777: 764: 760: 745: 741: 732: 717: 710: 706: 690: 682:fatty acid beta-oxidation 677:leucine catabolic process 585: 581: 569: 564: 555: 542: 491: 482: 429: 420: 390: 382: 378: 361: 348: 311: 290: 281: 277: 260: 247: 210: 187: 178: 174: 129: 126: 116: 109: 104: 77: 72: 55: 50: 45: 41: 37: 32: 3526:Diffusion-limited enzyme 3358:Porphobilinogen synthase 3104:Methylmalonyl-CoA mutase 2681:D-cysteine desulfhydrase 2297:Medical Subject Headings 2238:Kohlmeier M (May 2015). 1090:. It is a member of the 1006:Chr 13: 52.99 – 53.08 Mb 2835:Glutamate dehydrogenase 2810:Ornithine decarboxylase 2738:Histidine ammonia-lyase 2481:Leucine 2,3-aminomutase 1787:3-methylglutaconyl-CoA 999:Chr 9: 91.21 – 91.36 Mb 523:myocardium of ventricle 515:interventricular septum 3414:: Acting on phosphates 3222:Aspartate transaminase 3045:conversion to cysteine 3015:Adenosylhomocysteinase 2868:Glutathione synthetase 2149:10.1186/1550-2783-10-6 2084:Nucleic Acids Research 1462:β-Methylglutaconyl-CoA 1201:3-methylglutaconyl-CoA 1199:the transformation of 3619:Eadie–Hofstee diagram 3552:Allosteric regulation 3217:Asparagine synthetase 2385:Essential amino acids 1740:Clinical significance 1306:acid aminotransferase 1118:The enzyme AUH has a 306:Chromosome 13 (mouse) 3629:Lineweaver–Burk plot 2888:Glutamine synthetase 2662:Alanine transaminase 1453:β-Methylcrotonyl-CoA 1304:Branched-chain amino 1213:mitochondrial matrix 646:mitochondrial matrix 495:facial motor nucleus 465:right adrenal cortex 204:Chromosome 9 (human) 73:List of PDB id codes 46:Available structures 3445:Hydroperoxide lyase 3348:Tryptophan synthase 3334:Enoyl-CoA hydratase 3028:Methionine synthase 2941:Enoyl-CoA hydratase 2826:alpha-ketoglutarate 2743:Urocanate hydratase 2456:Enoyl-CoA hydratase 1750:leukoencephalopathy 1558:Enoyl-CoA hydratase 1184:enoyl-CoA hydratase 625:mRNA 3'-UTR binding 469:left adrenal cortex 3588:Enzyme superfamily 3521:Enzyme promiscuity 3421:Threonine synthase 3302:Carbonic anhydrase 3074:Threonine aldolase 2623:Serine dehydratase 2367:Protein metabolism 2096:10.1093/nar/gku179 1994:10.1002/prot.22246 812:ENSMUSG00000021460 660:Biological process 639:Cellular component 610:catalytic activity 593:Molecular function 3744: 3743: 3453: 3452: 3397:Hyaluronate lyase 3378:Nitrile hydratase 3238: 3237: 3234: 3233: 3230: 3229: 3184: 3183: 3112: 3111: 2901: 2900: 2708: 2707: 2575: 2574: 2466:HMG-CoA reductase 2255:978-0-12-387784-0 1733: 1732: 1696:metabolic pathway 1644:β-Hydroxybutyrate 1612:β-Hydroxybutyrate 1364:α-Ketoisocaproate 1352:α-Ketoisocaproate 1241:protein synthesis 1187:presence of RNA. 1079:) encoded by the 1055: 1054: 1051: 1050: 1024: 1023: 989: 988: 968: 967: 926: 925: 905: 904: 863: 862: 844: 843: 818: 817: 799: 798: 773: 772: 754: 753: 702: 701: 577: 576: 573: 572: 551: 550: 538: 537: 476: 475: 374: 373: 345:13|13 A5- B1 273: 272: 100: 99: 96: 95: 56:Ortholog search: 16:(Redirected from 3791: 3761: 3760: 3752: 3624:Hanes–Woolf plot 3567:Enzyme activator 3562:Enzyme inhibitor 3536:Enzyme catalysis 3480: 3473: 3466: 3457: 3456: 3279:4.2) (primarily 3265: 3258: 3251: 3242: 3241: 3196: 3195: 3124: 3123: 2918: 2917: 2725: 2724: 2601: 2600: 2586: 2585: 2404: 2403: 2356: 2349: 2342: 2333: 2332: 2281: 2278: 2277: 2270: 2264: 2262: 2235: 2226: 2225: 2196:(4): 1015–1025. 2181: 2172: 2171: 2161: 2151: 2127: 2118: 2117: 2107: 2075: 2066: 2065: 2047: 2032:The FEBS Journal 2023: 2014: 2013: 1977: 1966: 1965: 1955: 1931: 1925: 1924: 1896: 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3794: 3793: 3792: 3790: 3789: 3788: 3769: 3768: 3767: 3755: 3747: 3745: 3740: 3652:Oxidoreductases 3638: 3614:Enzyme kinetics 3602: 3598:List of enzymes 3571: 3540: 3511:Catalytic triad 3489: 3484: 3454: 3449: 3425: 3406: 3382: 3284: 3269: 3239: 3226: 3180: 3108: 3078: 3059: 2989: 2960: 2911: 2897: 2839: 2819: 2790: 2767:Proline oxidase 2752: 2720:α-ketoglutarate 2718: 2704: 2685: 2666: 2647: 2643:Creatine kinase 2593: 2571: 2550: 2526:Arylformamidase 2502: 2426: 2389: 2387:are in Capitals 2377: 2373:and catabolism 2360: 2289: 2284: 2275: 2274: 2271: 2260: 2258: 2256: 2236: 2229: 2182: 2175: 2128: 2121: 2076: 2069: 2024: 2017: 1978: 1969: 1946:(12): 1253–63. 1932: 1928: 1897: 1890: 1881: 1880: 1876: 1863: 1862: 1858: 1845: 1844: 1840: 1828: 1819: 1807: 1798: 1794: 1778: 1742: 1736: 1734: 1729: 1728: 1727: 1720: 1719: 1718: 1710: 1709: 1708: 1693: 1685: 1684: 1683: 1682: 1677: 1675: 1670: 1668: 1663: 1661: 1656: 1654: 1651:Acetoacetyl-CoA 1649: 1647: 1642: 1640: 1637: 1635: 1633: 1628: 1626: 1622: 1619: 1617: 1613: 1610: 1608: 1604: 1601: 1599: 1595: 1592: 1590: 1585: 1582: 1581: 1579: 1575: 1572: 1570: 1566: 1563: 1561: 1556: 1554: 1550: 1547: 1541: 1539: 1535: 1530: 1528: 1525: 1520: 1518: 1514: 1509: 1507: 1504: 1499: 1497: 1494: 1489: 1487: 1484: 1479: 1477: 1474: 1469: 1467: 1464: 1460: 1458: 1455: 1451: 1449: 1446: 1442: 1440: 1435: 1432: 1430: 1427: 1425: 1421: 1419: 1412: 1408: 1404: 1402: 1397: 1395: 1388: 1386:KIC-dioxygenase 1384: 1382: 1373: 1370: 1368: 1358: 1356: 1346: 1344: 1339: 1337: 1332: 1330: 1325: 1323: 1318: 1316: 1313:α-Ketoglutarate 1311: 1309: 1305: 1302: 1300: 1292: 1291: 1290: 1288: 1284: 1276: 1221:AU-rich element 1195:AUH is seen to 1193: 1140:skeletal muscle 1116: 1046:View/Edit Mouse 1041:View/Edit Human 1004: 997: 994:Location (UCSC) 980: 959: 955: 951: 947: 943: 917: 896: 892: 888: 884: 880: 793:ENSG00000148090 686: 655: 634: 543: 534: 529: 527:muscle of thigh 525: 521: 519:proximal tubule 517: 513: 509: 505: 503:right ventricle 501: 497: 483: 472: 467: 463: 459: 455: 451: 447: 445:right ventricle 443: 439: 435: 421: 365: 352: 344: 334: 333: 332: 325: 305: 282:Gene location ( 264: 251: 243: 233: 232: 231: 224: 202: 179:Gene location ( 168:AUH - orthologs 130: 117: 110: 79: 57: 28: 23: 22: 15: 12: 11: 5: 3797: 3787: 3786: 3781: 3766: 3765: 3742: 3741: 3739: 3738: 3725: 3712: 3699: 3686: 3673: 3660: 3646: 3644: 3640: 3639: 3637: 3636: 3631: 3626: 3621: 3616: 3610: 3608: 3604: 3603: 3601: 3600: 3595: 3590: 3585: 3579: 3577: 3576:Classification 3573: 3572: 3570: 3569: 3564: 3559: 3554: 3548: 3546: 3542: 3541: 3539: 3538: 3533: 3528: 3523: 3518: 3513: 3508: 3503: 3497: 3495: 3491: 3490: 3483: 3482: 3475: 3468: 3460: 3451: 3450: 3448: 3447: 3442: 3436: 3434: 3427: 3426: 3424: 3423: 3417: 3415: 3408: 3407: 3405: 3404: 3399: 3393: 3391: 3384: 3383: 3381: 3380: 3375: 3370: 3365: 3360: 3355: 3350: 3345: 3340: 3331: 3330: 3329: 3324: 3314: 3309: 3304: 3298: 3296: 3286: 3285: 3271:Carbon–oxygen 3268: 3267: 3260: 3253: 3245: 3236: 3235: 3232: 3231: 3228: 3227: 3225: 3224: 3219: 3209: 3207: 3193: 3186: 3185: 3182: 3181: 3179: 3178: 3164: 3163: 3158: 3153: 3148: 3143: 3137: 3135: 3121: 3114: 3113: 3110: 3109: 3107: 3106: 3101: 3096: 3090: 3088: 3080: 3079: 3077: 3076: 3070: 3068: 3061: 3060: 3058: 3057: 3052: 3040: 3039: 3034: 3018: 3017: 3012: 3000: 2998: 2991: 2990: 2988: 2987: 2982: 2977: 2971: 2969: 2962: 2961: 2959: 2958: 2953: 2948: 2943: 2938: 2933: 2927: 2925: 2915: 2903: 2902: 2899: 2898: 2896: 2895: 2890: 2876: 2875: 2870: 2865: 2847: 2845: 2841: 2840: 2838: 2837: 2831: 2829: 2821: 2820: 2818: 2817: 2812: 2807: 2801: 2799: 2792: 2791: 2789: 2788: 2783: 2774: 2769: 2763: 2761: 2754: 2753: 2751: 2750: 2745: 2740: 2734: 2732: 2722: 2710: 2709: 2706: 2705: 2703: 2702: 2696: 2694: 2687: 2686: 2684: 2683: 2677: 2675: 2668: 2667: 2665: 2664: 2658: 2656: 2649: 2648: 2646: 2645: 2640: 2626: 2625: 2620: 2614: 2612: 2598: 2583: 2577: 2576: 2573: 2572: 2570: 2569: 2565: 2563: 2552: 2551: 2549: 2548: 2543: 2538: 2533: 2528: 2523: 2513: 2511: 2504: 2503: 2494: 2493: 2488: 2483: 2478: 2473: 2468: 2463: 2458: 2453: 2448: 2443: 2437: 2435: 2428: 2427: 2425: 2424: 2419: 2413: 2411: 2401: 2391: 2390: 2382: 2379: 2378: 2359: 2358: 2351: 2344: 2336: 2330: 2329: 2308: 2300: 2288: 2287:External links 2285: 2283: 2282: 2254: 2227: 2173: 2119: 2090:(9): 5483–94. 2067: 2038:(9): 2012–22. 2015: 1967: 1926: 1888: 1874: 1856: 1838: 1817: 1795: 1793: 1790: 1789: 1788: 1785: 1777: 1774: 1741: 1738: 1731: 1730: 1704:isovaleryl-CoA 1687: 1686: 1565:Isovaleryl-CoA 1533: 1523: 1512: 1502: 1492: 1482: 1472: 1399:Isovaleryl-CoA 1286: 1285: 1278: 1277: 1272: 1271: 1270: 1265: 1215:. Also, these 1192: 1189: 1120:molecular mass 1115: 1112: 1053: 1052: 1049: 1048: 1043: 1033: 1032: 1026: 1025: 1022: 1021: 1019: 1017: 1010: 1009: 1002: 995: 991: 990: 987: 986: 976: 975: 969: 966: 965: 939: 938: 932: 928: 927: 924: 923: 913: 912: 906: 903: 902: 876: 875: 869: 865: 864: 861: 860: 852: 851: 845: 842: 841: 833: 832: 826: 820: 819: 816: 815: 807: 806: 800: 797: 796: 788: 787: 781: 775: 774: 771: 770: 762: 761: 755: 752: 751: 743: 742: 736: 730: 729: 724: 719: 715: 714: 704: 703: 700: 699: 688: 687: 685: 684: 679: 674: 669: 663: 661: 657: 656: 654: 653: 648: 642: 640: 636: 635: 633: 632: 627: 622: 617: 615:lyase activity 612: 607: 602: 596: 594: 590: 589: 579: 578: 575: 574: 571: 570: 567: 566: 559: 553: 552: 549: 548: 540: 539: 536: 535: 533: 532: 528: 524: 520: 516: 512: 508: 504: 500: 496: 492: 489: 488: 477: 474: 473: 471: 470: 466: 462: 458: 454: 450: 446: 442: 438: 434: 430: 427: 426: 414: 413: 405: 394: 388: 387: 384:RNA expression 376: 375: 372: 371: 363: 359: 358: 350: 347: 342: 336: 335: 326: 319: 313: 309: 308: 303: 297: 296: 288: 287: 275: 274: 271: 270: 262: 258: 257: 249: 246: 241: 235: 234: 225: 218: 212: 208: 207: 200: 194: 193: 185: 184: 172: 171: 128: 124: 123: 115: 107: 106: 102: 101: 98: 97: 94: 93: 75: 74: 66: 65: 54: 48: 47: 39: 38: 35: 34: 26: 9: 6: 4: 3: 2: 3796: 3785: 3782: 3780: 3777: 3776: 3774: 3764: 3759: 3754: 3753: 3750: 3736: 3732: 3731: 3726: 3723: 3719: 3718: 3713: 3710: 3706: 3705: 3700: 3697: 3693: 3692: 3687: 3684: 3680: 3679: 3674: 3671: 3667: 3666: 3661: 3658: 3654: 3653: 3648: 3647: 3645: 3641: 3635: 3632: 3630: 3627: 3625: 3622: 3620: 3617: 3615: 3612: 3611: 3609: 3605: 3599: 3596: 3594: 3593:Enzyme family 3591: 3589: 3586: 3584: 3581: 3580: 3578: 3574: 3568: 3565: 3563: 3560: 3558: 3557:Cooperativity 3555: 3553: 3550: 3549: 3547: 3543: 3537: 3534: 3532: 3529: 3527: 3524: 3522: 3519: 3517: 3516:Oxyanion hole 3514: 3512: 3509: 3507: 3504: 3502: 3499: 3498: 3496: 3492: 3488: 3481: 3476: 3474: 3469: 3467: 3462: 3461: 3458: 3446: 3443: 3441: 3438: 3437: 3435: 3432: 3428: 3422: 3419: 3418: 3416: 3413: 3409: 3403: 3400: 3398: 3395: 3394: 3392: 3389: 3385: 3379: 3376: 3374: 3371: 3369: 3366: 3364: 3361: 3359: 3356: 3354: 3351: 3349: 3346: 3344: 3341: 3339: 3335: 3332: 3328: 3325: 3323: 3320: 3319: 3318: 3315: 3313: 3310: 3308: 3305: 3303: 3300: 3299: 3297: 3295: 3291: 3287: 3282: 3278: 3274: 3266: 3261: 3259: 3254: 3252: 3247: 3246: 3243: 3223: 3220: 3218: 3214: 3211: 3210: 3208: 3205: 3201: 3197: 3194: 3192: 3187: 3177: 3173: 3169: 3166: 3165: 3162: 3159: 3157: 3154: 3152: 3149: 3147: 3144: 3142: 3139: 3138: 3136: 3133: 3129: 3128:PHENYLALANINE 3125: 3122: 3120: 3115: 3105: 3102: 3100: 3097: 3095: 3092: 3091: 3089: 3086: 3081: 3075: 3072: 3071: 3069: 3066: 3062: 3056: 3053: 3051: 3048: 3046: 3042: 3041: 3038: 3035: 3033: 3029: 3026: 3024: 3020: 3019: 3016: 3013: 3011: 3008: 3006: 3002: 3001: 2999: 2996: 2992: 2986: 2983: 2981: 2978: 2976: 2973: 2972: 2970: 2967: 2963: 2957: 2954: 2952: 2949: 2947: 2944: 2942: 2939: 2937: 2934: 2932: 2929: 2928: 2926: 2923: 2919: 2916: 2914: 2909: 2908:propionyl-CoA 2904: 2894: 2891: 2889: 2885: 2881: 2878: 2877: 2874: 2871: 2869: 2866: 2864: 2860: 2856: 2852: 2849: 2848: 2846: 2842: 2836: 2833: 2832: 2830: 2827: 2822: 2816: 2813: 2811: 2808: 2806: 2803: 2802: 2800: 2797: 2793: 2787: 2784: 2782: 2778: 2775: 2773: 2770: 2768: 2765: 2764: 2762: 2759: 2755: 2749: 2746: 2744: 2741: 2739: 2736: 2735: 2733: 2730: 2726: 2723: 2721: 2716: 2711: 2701: 2698: 2697: 2695: 2692: 2688: 2682: 2679: 2678: 2676: 2673: 2669: 2663: 2660: 2659: 2657: 2654: 2650: 2644: 2641: 2639: 2635: 2631: 2628: 2627: 2624: 2621: 2619: 2616: 2615: 2613: 2610: 2606: 2602: 2599: 2597: 2592: 2587: 2584: 2582: 2578: 2567: 2566: 2564: 2561: 2557: 2556:PHENYLALANINE 2553: 2547: 2544: 2542: 2539: 2537: 2534: 2532: 2529: 2527: 2524: 2522: 2518: 2515: 2514: 2512: 2509: 2505: 2501: 2499: 2492: 2489: 2487: 2484: 2482: 2479: 2477: 2474: 2472: 2469: 2467: 2464: 2462: 2461:HMG-CoA lyase 2459: 2457: 2454: 2452: 2449: 2447: 2444: 2442: 2439: 2438: 2436: 2433: 2429: 2423: 2420: 2418: 2415: 2414: 2412: 2409: 2405: 2402: 2400: 2396: 2392: 2388: 2386: 2380: 2376: 2372: 2368: 2364: 2357: 2352: 2350: 2345: 2343: 2338: 2337: 2334: 2327: 2323: 2322: 2317: 2313: 2309: 2307: 2304: 2301: 2298: 2294: 2291: 2290: 2280: 2269: 2257: 2251: 2247: 2246: 2241: 2234: 2232: 2224: 2219: 2215: 2211: 2207: 2203: 2199: 2195: 2191: 2187: 2180: 2178: 2169: 2165: 2160: 2155: 2150: 2145: 2141: 2137: 2133: 2126: 2124: 2115: 2111: 2106: 2101: 2097: 2093: 2089: 2085: 2081: 2074: 2072: 2063: 2059: 2055: 2051: 2046: 2041: 2037: 2033: 2029: 2022: 2020: 2011: 2007: 2003: 1999: 1995: 1991: 1988:(2): 360–72. 1987: 1983: 1976: 1974: 1972: 1963: 1959: 1954: 1949: 1945: 1941: 1937: 1930: 1922: 1918: 1914: 1910: 1906: 1902: 1895: 1893: 1884: 1878: 1870: 1866: 1860: 1852: 1848: 1842: 1835: 1831: 1826: 1824: 1822: 1814: 1810: 1805: 1803: 1801: 1796: 1786: 1783: 1782: 1781: 1773: 1771: 1767: 1763: 1759: 1755: 1751: 1747: 1737: 1716: 1705: 1701: 1697: 1680: 1673: 1666: 1659: 1652: 1645: 1631: 1624: 1615: 1614:dehydrogenase 1606: 1603:HMG-CoA  1597: 1587: 1577: 1568: 1567:dehydrogenase 1559: 1552: 1545: 1537: 1526: 1516: 1505: 1495: 1485: 1475: 1463: 1454: 1445: 1438: 1433: 1424: 1416: 1411: 1410: 1400: 1392: 1387: 1379: 1375: 1374:dehydrogenase 1365: 1361: 1353: 1349: 1342: 1335: 1328: 1321: 1314: 1307: 1298: 1282: 1275: 1269: 1268: 1264: 1262: 1258: 1254: 1250: 1246: 1242: 1238: 1235:survival and 1234: 1230: 1226: 1222: 1218: 1214: 1209: 1206: 1202: 1198: 1188: 1185: 1181: 1177: 1173: 1169: 1165: 1161: 1157: 1153: 1149: 1145: 1141: 1137: 1133: 1129: 1125: 1121: 1111: 1109: 1105: 1101: 1097: 1093: 1089: 1088:chromosome 19 1085: 1082: 1078: 1075: 1071: 1067: 1063: 1059: 1047: 1042: 1038: 1034: 1031: 1027: 1020: 1018: 1015: 1011: 1007: 1003: 1000: 996: 992: 985: 983: 977: 973: 970: 964: 962: 958: 954: 950: 946: 940: 936: 933: 929: 922: 920: 914: 910: 907: 901: 899: 895: 891: 887: 883: 877: 873: 870: 868:RefSeq (mRNA) 866: 859: 858: 853: 849: 846: 840: 839: 834: 830: 827: 825: 821: 814: 813: 808: 804: 801: 795: 794: 789: 785: 782: 780: 776: 769: 768: 763: 759: 756: 750: 749: 744: 740: 737: 735: 731: 728: 725: 723: 720: 716: 713: 709: 705: 698: 694: 689: 683: 680: 678: 675: 673: 670: 668: 665: 664: 662: 659: 658: 652: 651:mitochondrion 649: 647: 644: 643: 641: 638: 637: 631: 628: 626: 623: 621: 618: 616: 613: 611: 608: 606: 603: 601: 598: 597: 595: 592: 591: 588: 587:Gene ontology 584: 580: 568: 563: 560: 558: 554: 546: 541: 530: 526: 522: 518: 514: 510: 506: 502: 498: 494: 493: 490: 486: 481: 478: 468: 464: 460: 457:parotid gland 456: 452: 448: 444: 441:kidney tubule 440: 436: 433:renal medulla 432: 431: 428: 424: 419: 416: 415: 412: 410: 406: 404: 403: 399: 398: 395: 393: 389: 385: 381: 377: 369: 364: 360: 356: 351: 341: 337: 330: 323: 317: 310: 302: 298: 294: 289: 285: 280: 276: 268: 263: 259: 255: 250: 240: 236: 229: 222: 216: 209: 205: 199: 195: 191: 186: 182: 177: 173: 169: 165: 161: 157: 153: 149: 145: 141: 137: 133: 125: 120: 113: 108: 103: 92: 90: 86: 82: 76: 71: 68: 67: 63: 60: 53: 49: 44: 40: 36: 31: 19: 3730:Translocases 3727: 3714: 3701: 3688: 3675: 3665:Transferases 3662: 3649: 3506:Binding site 3402:Pectin lyase 3342: 3294:Hydro-Lyases 3281:dehydratases 3213:Asparaginase 3191:oxaloacetate 3085:succinyl-CoA 3043: 3021: 3003: 2913:succinyl-CoA 2531:Kynureninase 2495: 2490: 2383: 2319: 2266: 2259:. Retrieved 2244: 2221: 2193: 2189: 2139: 2135: 2087: 2083: 2035: 2031: 1985: 1981: 1943: 1939: 1929: 1907:(3): 410–3. 1904: 1900: 1877: 1868: 1859: 1850: 1841: 1779: 1776:Interactions 1753: 1743: 1735: 1706:relative to 1665:Acetoacetate 1583: 1431: 1414: 1406: 1378:mitochondria 1194: 1117: 1080: 1065: 1061: 1057: 1056: 979: 961:NP_001338362 957:NP_001338361 953:NP_001338360 945:NP_001293119 942: 916: 898:NM_001351433 894:NM_001351432 890:NM_001351431 882:NM_001306190 879: 855: 836: 810: 791: 765: 746: 726: 721: 507:right kidney 453:human kidney 407: 400: 127:External IDs 78: 3501:Active site 2893:Glutaminase 2859:glutathione 2568:(see below) 2190:Amino Acids 1679:Cholesterol 1576:carboxylase 1257:cell growth 1229:degradation 1168:alpha helix 620:RNA binding 366:53,083,717 353:52,989,155 265:91,361,918 252:91,213,815 105:Identifiers 3773:Categories 3704:Isomerases 3678:Hydrolases 3545:Regulation 3200:asparagine 3176:Tyrosinase 2995:METHIONINE 2966:ISOLEUCINE 2815:Agmatinase 2508:TRYPTOPHAN 2399:acetyl-CoA 2363:Metabolism 1836:, May 2017 1815:, May 2017 1792:References 1672:Mevalonate 1658:Acetyl-CoA 1621:Mevalonate 1253:morphology 1249:biogenesis 1245:metabolism 1237:transcript 1225:nucleotide 1217:reductions 1208:catabolism 1176:oligomeric 1170:H1. These 672:metabolism 411:(ortholog) 148:HomoloGene 18:AUH (gene) 3583:EC number 3368:Urocanase 3327:Enolase 2 3312:Aconitase 3204:aspartate 3065:THREONINE 2884:glutamine 2880:glutamate 2855:glutamate 2729:HISTIDINE 2715:glutamate 2691:threonine 2371:synthesis 2240:"Leucine" 1940:Structure 1596:reductase 1586:hydratase 1447:(HMG-CoA) 1407:β-Hydroxy 1327:Glutamate 1320:Glutamate 1130:, is 1.7 1114:Structure 1108:Mutations 1100:metabolic 982:NP_057918 949:NP_001689 919:NM_016709 886:NM_001698 712:Orthologs 156:GeneCards 3784:EC 4.2.1 3607:Kinetics 3531:Cofactor 3494:Activity 3307:Fumarase 3168:tyrosine 3132:tyrosine 3119:fumarate 2851:cysteine 2796:arginine 2672:cysteine 2634:creatine 2591:pyruvate 2560:tyrosine 2306:4.2.1.18 2279:-leucine 2218:11120110 2210:20607321 2168:23374455 2142:(1): 6. 2114:24598254 2054:16640564 2010:44523407 2002:18831052 1982:Proteins 1962:11738050 1921:21840233 1832:– 1811:– 1762:splicing 1758:missense 1724:-leucine 1714:-leucine 1630:Thiolase 1605:synthase 1465:(MG-CoA) 1456:(MC-CoA) 1428:(10–40%) 1426:in urine 1423:Excreted 1341:Pyruvate 1296:-Leucine 1197:catalyze 1191:Function 1077:4.2.1.18 1068:, is an 1030:Wikidata 691:Sources: 3763:Biology 3717:Ligases 3487:Enzymes 3433:: Other 3317:Enolase 3172:melanin 2781:ALDH4A1 2758:proline 2653:alanine 2630:glycine 2605:glycine 2596:citrate 2432:LEUCINE 2375:enzymes 2326:PDBe-KB 2316:UniProt 2159:3568064 2105:4027184 2062:6261362 1834:Ensembl 1813:Ensembl 1636:Unknown 1623:pathway 1594:HMG-CoA 1549:HMG-CoA 1437:HMB-CoA 1391:cytosol 1366:(α-KIC) 1354:(α-KIC) 1334:Alanine 1205:leucine 1164:trimers 1156:hexamer 1104:protein 824:UniProt 779:Ensembl 718:Species 697:QuickGO 386:pattern 244:9q22.31 144:1338011 112:Aliases 3749:Portal 3691:Lyases 3431:4.2.99 3273:lyases 2922:VALINE 2609:serine 2408:LYSINE 2321:Q13825 2299:(MeSH) 2261:6 June 2252:  2216:  2208:  2166:  2156:  2112:  2102:  2060:  2052:  2008:  2000:  1960:  1919:  1768:and 1 1694:Human 1638:enzyme 1584:MG-CoA 1574:MC-CoA 1348:Muscle 1233:neuron 1172:lysine 1152:spleen 1150:, and 1136:kidney 1122:of 32 1070:enzyme 1016:search 1014:PubMed 857:Q9JLZ3 838:Q13825 734:Entrez 557:BioGPS 499:zygote 136:600529 3643:Types 3412:4.2.3 3388:4.2.2 3322:Alpha 3290:4.2.1 2844:Other 2786:PYCR1 2496:(See 2214:S2CID 2058:S2CID 2006:S2CID 1551:lyase 1544:liver 1360:Liver 1160:dimer 1158:as a 1148:liver 1144:heart 1128:exons 767:11992 727:Mouse 722:Human 693:Amigo 409:Mouse 402:Human 349:Start 284:Mouse 248:Start 181:Human 3735:list 3728:EC7 3722:list 3715:EC6 3709:list 3702:EC5 3696:list 3689:EC4 3683:list 3676:EC3 3670:list 3663:EC2 3657:list 3650:EC1 3087:→TCA 2828:→TCA 2314:for 2263:2016 2250:ISBN 2206:PMID 2164:PMID 2110:PMID 2050:PMID 1998:PMID 1958:PMID 1917:PMID 1784:ARE 1764:, 1 1760:, 3 1702:and 1698:for 1084:gene 1064:and 449:pons 392:Bgee 340:Band 301:Chr. 239:Band 198:Chr. 152:1284 132:OMIM 89:2ZQR 85:2ZQQ 81:1HZD 62:RCSB 59:PDBe 2312:PDB 2198:doi 2154:PMC 2144:doi 2100:PMC 2092:doi 2040:doi 2036:273 1990:doi 1948:doi 1909:doi 1905:104 1754:AUH 1700:HMB 1415:HMB 1162:of 1124:kDa 1096:RNA 1086:on 1081:AUH 1066:AUH 748:549 565:n/a 362:End 261:End 164:OMA 160:AUH 140:MGI 119:AUH 52:PDB 33:AUH 3775:: 3292:: 3277:EC 3189:G→ 3174:: 3117:G→ 2906:G→ 2886:: 2861:: 2713:G→ 2636:: 2589:G→ 2369:, 2365:: 2318:: 2303:EC 2265:. 2242:. 2230:^ 2220:. 2212:. 2204:. 2194:40 2192:. 2188:. 2176:^ 2162:. 2152:. 2140:10 2138:. 2134:. 2122:^ 2108:. 2098:. 2088:42 2086:. 2082:. 2070:^ 2056:. 2048:. 2034:. 2030:. 2018:^ 2004:. 1996:. 1986:75 1984:. 1970:^ 1956:. 1942:. 1938:. 1915:. 1903:. 1891:^ 1867:. 1849:. 1820:^ 1799:^ 1522:CO 1501:CO 1481:CO 1471:CO 1362:: 1350:: 1251:, 1247:, 1146:, 1142:, 1138:, 1132:kb 1106:. 1074:EC 695:/ 368:bp 355:bp 267:bp 254:bp 162:; 158:: 154:; 150:: 146:; 142:: 138:; 134:: 87:, 83:, 3751:: 3737:) 3733:( 3724:) 3720:( 3711:) 3707:( 3698:) 3694:( 3685:) 3681:( 3672:) 3668:( 3659:) 3655:( 3479:e 3472:t 3465:v 3336:/ 3283:) 3275:( 3264:e 3257:t 3250:v 3215:/ 3206:→ 3202:→ 3170:→ 3134:→ 3130:→ 3083:→ 3067:→ 3047:: 3030:/ 3025:: 3007:: 2997:→ 2968:→ 2924:→ 2910:→ 2882:→ 2857:→ 2853:+ 2824:→ 2798:→ 2779:/ 2760:→ 2731:→ 2717:→ 2693:→ 2674:→ 2655:→ 2632:→ 2611:→ 2607:→ 2594:→ 2581:G 2562:→ 2558:→ 2519:/ 2510:→ 2434:→ 2410:→ 2397:→ 2395:K 2355:e 2348:t 2341:v 2328:. 2276:L 2200:: 2170:. 2146:: 2116:. 2094:: 2064:. 2042:: 2012:. 1992:: 1964:. 1950:: 1944:9 1923:. 1911:: 1885:. 1871:. 1853:. 1721:L 1711:L 1546:) 1542:( 1536:O 1534:2 1532:H 1524:2 1515:O 1513:2 1511:H 1503:2 1493:2 1491:O 1483:2 1473:2 1417:) 1413:( 1393:) 1389:( 1380:) 1376:( 1293:L 1072:( 286:) 183:) 166:: 20:)

Index

AUH (gene)
PDB
PDBe
RCSB
1HZD
2ZQQ
2ZQR
Aliases
AUH
OMIM
600529
MGI
1338011
HomoloGene
1284
GeneCards
AUH
OMA
AUH - orthologs
Human
Chromosome 9 (human)
Chr.
Chromosome 9 (human)
Chromosome 9 (human)
Genomic location for AUH
Genomic location for AUH
Band
bp
bp
Mouse

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