1301:. It is characterized by the buildup of a chemical called lactic acid in the body and a variety of neurological problems. Signs and symptoms of this condition usually first appear shortly after birth, and they can vary widely among affected individuals. The most common feature is a potentially life-threatening buildup of lactic acid (lactic acidosis), which can cause nausea, vomiting, severe breathing problems, and an abnormal heartbeat. People with pyruvate dehydrogenase deficiency usually have neurological problems as well. Most have delayed development of mental abilities and motor skills such as sitting and walking. Other neurological problems can include intellectual disability, seizures, weak muscle tone (hypotonia), poor coordination, and difficulty walking. Some affected individuals have abnormal brain structures, such as underdevelopment of the tissue connecting the left and right halves of the brain (corpus callosum), wasting away (atrophy) of the exterior part of the brain known as the cerebral cortex, or patches of damaged tissue (lesions) on some parts of the brain. Because of the severe health effects, many individuals with pyruvate dehydrogenase deficiency do not survive past childhood, although some may live into adolescence or adulthood.
1252:. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. The overall complex furthers catalysis by keeping the necessary substrates for the reaction close within the enzyme, thus creating a situation in which it is more likely that the substrate will be in the favorable conformation and orientation. This enzyme is also part of a larger multienzyme complex that channels the intermediates in the catalysis between subunits of the complex thus minimizing unwanted side reactions. Not only do the subunits ferry products back and forth, but each of the subunits in the E1o homodimer are connected via a cavity lined with acidic residues, thus increasing the dimer's ability to act as a base. The orientation of the cavity allows for direct transfer of the intermediate to the E2o subunit.
302:
279:
2655:
176:
201:
308:
207:
29:
1260:
The protein encoded by OGDH is thought to have a single active site. The enzyme also requires two cofactors in order for it to function properly, Thiamine diphosphate and a divalent magnesium ion. The specific mechanism of the subunit is currently unknown; however, there are several theories as to
1223:
This gene encodes a subunit that catalyzes the oxidative decarboxylation of alpha-ketoglutarate to
Succinyl-CoA at its active site in the fourth step of the citric acid cycle by acting as a base to facilitate the decarboxylation. The main residues responsible for the catalysis are thought to be His
2445:
Kimura K, Wakamatsu A, Suzuki Y, Ota T, Nishikawa T, Yamashita R, Yamamoto J, Sekine M, Tsuritani K, Wakaguri H, Ishii S, Sugiyama T, Saito K, Isono Y, Irie R, Kushida N, Yoneyama T, Otsuka R, Kanda K, Yokoi T, Kondo H, Wagatsuma M, Murakawa K, Ishida S, Ishibashi T, Takahashi-Fujii A, Tanase T,
2265:
Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P,
55:
2654:
1261:
how it functions, among them is the Hexa Uni Ping Pong theory. Even though the mechanism isn't fully known the kinetic data have been calculated and are as follows: the Km is 0.14 ± 0.04 mM, and the Vmax is 9 ± 3 μmol/(min*mg).
2266:
Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (Oct 2005). "Towards a proteome-scale map of the human protein-protein interaction network".
1775:
1772:
1760:
1740:
1736:
1379:
1376:
1364:
1344:
1340:
1770:
1374:
2491:"Subunit interactions in the mammalian alpha-ketoglutarate dehydrogenase complex. Evidence for direct association of the alpha-ketoglutarate dehydrogenase and dihydrolipoamide dehydrogenase components"
2524:
van Bever Y, Balemans W, Duval EL, Jespers A, Eyskens F, van Hul W, Courtens W (Apr 2007). "Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome".
1722:
1326:
1773:
1764:
1734:
1733:
1729:
1377:
1368:
1338:
1337:
1333:
2695:
3012:
2827:
315:
214:
2904:
2751:
2726:
2663:: Different thermodynamic binding mechanisms and peptide fine specificities associated with a panel of structurally similar high-affinity T cell receptors
1277:
is ΔG = −33 kJ/mol. The significant energy change makes it a crucial point of regulation not only for the citric acid cycle, but also for the entire
2688:
2599:
837:
137:
818:
1737:
1341:
1752:
1356:
2448:"Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes"
2681:
2634:
2112:
1912:
1894:
3150:
1768:
1755:
1741:
1372:
1359:
1345:
301:
3097:
2897:
2416:
Koike K (Jul 1995). "The gene encoding human 2-oxoglutarate dehydrogenase: structural organization and mapping to chromosome 7p13-p14".
1991:"Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2"
1771:
1375:
1215:
The OGDH gene is located on the 7th chromosome, with the specific location being 7p14-p13. There are 26 exons located within the gene.
2990:
2817:
2773:
1125:
278:
2744:
2722:
1132:
2037:
1757:
1361:
1778:
1750:
1725:
1382:
1354:
1329:
3155:
3055:
1881:
1860:
1763:
1726:
1367:
1330:
3060:
3032:
3005:
2983:
2968:
2933:
2890:
2847:
1877:
2232:"Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity"
200:
175:
52:
1765:
1730:
1369:
1334:
1856:
2978:
1781:
1780:
1739:
1727:
1385:
1384:
1343:
1331:
117:
1749:
1353:
1779:
1732:
1383:
1336:
2563:"Regulation of 2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability by the RING finger ubiquitin ligase Siah"
1766:
1370:
1784:
1767:
1761:
1756:
1754:
1746:
1388:
1371:
1365:
1360:
1358:
1350:
314:
213:
2739:
1785:
1782:
1745:
1389:
1386:
1349:
1233:
2177:
Leung PS, Rossaro L, Davis PA, Park O, Tanaka A, Kikuchi K, Miyakawa H, Norman GL, Lee W, Gershwin ME (Nov 2007).
1776:
1380:
2859:
2805:
2783:
2761:
2718:
2627:
307:
206:
2056:"Crystal structure of the E1 component of the Escherichia coli 2-oxoglutarate dehydrogenase multienzyme complex"
1787:
1786:
1777:
1747:
1742:
1391:
1390:
1381:
1351:
1346:
3082:
2709:
1783:
1769:
1751:
1744:
1735:
1731:
1728:
1387:
1373:
1355:
1348:
1339:
1335:
1332:
882:
125:
2863:
1774:
1758:
1753:
1748:
1743:
1378:
1362:
1357:
1352:
1347:
1762:
1759:
1738:
1366:
1363:
1342:
3114:
3092:
3072:
1237:
863:
3077:
2973:
2822:
2734:
1806:
1281:
pathway. As such, E1o is inhibited by both NADH and
Succinyl-CoA via non competitive feedback inhibition.
2620:
2275:
1943:
1932:"Cloning and nucleotide sequence of the cDNA encoding human 2-oxoglutarate dehydrogenase (lipoamide)"
1278:
189:
104:
3129:
3119:
3087:
1108:
1104:
1100:
1096:
1082:
1078:
1074:
1070:
1055:
1047:
1043:
1017:
1013:
1009:
1005:
991:
987:
983:
979:
964:
956:
952:
3124:
2561:
Habelhah H, Laine A, Erdjument-Bromage H, Tempst P, Gershwin ME, Bowtell DD, Ronai Z (Dec 2004).
2549:
2299:
2179:"Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis"
2130:"Studies on the mechanism and kinetics of the 2-oxoglutarate dehydrogenase system from pig heart"
1294:
149:
1086:
1051:
995:
960:
2871:
2584:
2541:
2512:
2477:
2433:
2404:
2369:
2334:
2291:
2253:
2208:
2159:
2108:
2085:
2012:
1971:
1274:
1270:
1249:
97:
45:
601:
oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
2795:
2574:
2533:
2502:
2467:
2459:
2425:
2394:
2359:
2324:
2283:
2243:
2198:
2190:
2149:
2141:
2075:
2067:
2002:
1961:
1951:
394:
325:
269:
224:
2917:
1298:
369:
145:
2673:
2279:
1947:
1289:
A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to
3065:
2948:
2925:
2705:
2472:
2447:
2203:
2178:
2154:
2129:
2080:
2055:
2329:
2312:
752:
747:
742:
737:
732:
727:
722:
717:
712:
707:
702:
697:
692:
687:
682:
677:
661:
656:
651:
646:
641:
636:
620:
615:
610:
605:
600:
595:
590:
585:
3144:
2960:
2943:
2603:
2429:
1966:
1931:
1827:
1810:
1797:
572:
2553:
129:
2346:
Sanger Centre, The; Washington
University Genome Sequencing Cente, The (Nov 1998).
2303:
1241:
387:
166:
2882:
153:
2938:
2489:
McCartney RG, Rice JE, Sanderson SJ, Bunik V, Lindsay H, Lindsay JG (Sep 1998).
1917:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1899:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1936:
Proceedings of the
National Academy of Sciences of the United States of America
470:
3050:
3027:
2842:
2765:
2103:
Voet DJ, Voet JG, Pratt CW (2010). "Chapter 18, Mitochondrial ATP synthesis".
2071:
1310:
Click on genes, proteins and metabolites below to link to respective articles.
286:
183:
133:
2507:
2490:
2809:
2383:"Normalization and subtraction: two approaches to facilitate gene discovery"
1290:
782:
530:
408:
353:
340:
252:
239:
141:
2588:
2579:
2562:
2545:
2481:
2295:
2257:
2248:
2231:
2212:
2089:
2038:"Entrez Gene: oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)"
2007:
1990:
1956:
2516:
2437:
2408:
2373:
2338:
2016:
1975:
1721:
1325:
1172:
1167:
28:
2537:
2364:
2347:
2163:
1156:
927:
908:
2287:
2463:
2399:
2382:
2313:"Structure-function relationships in dihydrolipoamide acyltransferases"
894:
849:
698:
tangential migration from the subventricular zone to the olfactory bulb
2194:
2145:
74:
3022:
3017:
2913:
2837:
2832:
2787:
1192:
1140:
804:
3107:
3102:
1269:
This subunit, known as E1o, catalyzes a rate-limiting step in the
108:, AKGDH, E1k, OGDC, oxoglutarate dehydrogenase, KGD1, OGDH2, OGDHD
2054:
Frank RA, Price AJ, Northrop FD, Perham RN, Luisi BF (May 2007).
767:
763:
3000:
1199:
121:
2886:
2677:
2616:
2612:
2446:
Nagai K, Kikuchi H, Nakai K, Isogai T, Sugano S (Jan 2006).
1720:
1324:
1825:
The interactive pathway map can be edited at WikiPathways:
606:
oxoglutarate dehydrogenase (succinyl-transferring) activity
1236:
complex. This complex catalyzes the overall conversion of
1189:
2-oxoglutarate dehydrogenase E1 component, mitochondrial
377:
542:
3013:
Branched-chain alpha-keto acid dehydrogenase complex
2828:
Branched-chain alpha-keto acid dehydrogenase complex
1224:
260, Phe 227, Gln685, His 729, Ser302, and His 298.
3115:
Phosphoenolpyruvate sugar phosphotransferase system
3043:
2959:
2924:
2858:
2804:
2782:
2760:
2717:
1273:and lies far from equilibrium; the total change in
1069:
1036:
978:
945:
1873:
1871:
1869:
1852:
1850:
1848:
324:
223:
2934:Photosynthetic reaction center complex proteins
2049:
2047:
1724:
1328:
2752:Mycothiol-dependent formaldehyde dehydrogenase
1878:GRCm38: Ensembl release 89: ENSMUSG00000020456
693:generation of precursor metabolites and energy
2898:
2689:
2628:
8:
2381:Bonaldo MF, Lennon G, Soares MB (Sep 1996).
2230:Shi Q, Chen HL, Xu H, Gibson GE (Mar 2005).
1989:Szabo P, Cai X, Ali G, Blass JP (Mar 1994).
683:olfactory bulb mitral cell layer development
2526:American Journal of Medical Genetics Part A
1857:GRCh38: Ensembl release 89: ENSG00000105953
2905:
2891:
2883:
2696:
2682:
2674:
2635:
2621:
2613:
2600:United States National Library of Medicine
1066:
975:
778:
586:oxoglutarate dehydrogenase (NAD+) activity
568:
451:Skeletal muscle tissue of rectus abdominis
365:
264:
161:
63:
2578:
2506:
2471:
2398:
2363:
2348:"Toward a complete human genome sequence"
2328:
2247:
2202:
2153:
2079:
2006:
1965:
1955:
2598:This article incorporates text from the
2032:
2030:
2028:
2026:
2650:
1844:
1818:
1232:This gene encodes one subunit of the 2-
485:cardiac muscle tissue of left ventricle
18:
1930:Koike K, Urata Y, Goto S (Mar 1992).
329:
290:
285:
228:
187:
182:
7:
2107:(4th ed.). Wiley. p. 669.
3098:Mitochondrial trifunctional protein
2567:The Journal of Biological Chemistry
2495:The Journal of Biological Chemistry
2317:The Journal of Biological Chemistry
2236:The Journal of Biological Chemistry
2818:Oxoglutarate dehydrogenase complex
2774:Formate dehydrogenase (cytochrome)
2128:McMinn CL, Ottaway JH (Mar 1977).
1033:
942:
918:
899:
873:
854:
828:
809:
657:oxoglutarate dehydrogenase complex
547:
465:
403:
382:
14:
2745:Long-chain-aldehyde dehydrogenase
1195:that in humans is encoded by the
1185:Alpha-ketoglutarate dehydrogenase
2653:
2311:Reed LJ, Hackert ML (Jun 1990).
733:2-oxoglutarate metabolic process
505:extensor digitorum longus muscle
313:
306:
300:
277:
212:
205:
199:
174:
27:
3056:Carbamoyl phosphate synthase II
16:Enzyme involved in Kreb's cycle
3061:Aspartate carbamoyltransferase
2969:Pyruvate dehydrogenase complex
738:succinyl-CoA metabolic process
611:thiamine pyrophosphate binding
531:More reference expression data
1:
3093:Glycine decarboxylase complex
3088:Fatty acid synthetase complex
2330:10.1016/S0021-9258(19)38795-2
708:cerebellar cortex development
298:
197:
2430:10.1016/0378-1119(95)00086-L
2060:Journal of Molecular Biology
713:pyramidal neuron development
443:myocardium of left ventricle
3151:Genes on human chromosome 7
3172:
3125:Sucrase-isomaltase complex
2991:Oxoglutarate dehydrogenase
2740:Acetaldehyde dehydrogenase
2105:Principles of Biochemistry
1234:oxoglutarate dehydrogenase
616:heat shock protein binding
2648:
2072:10.1016/j.jmb.2007.01.080
1913:"Mouse PubMed Reference:"
1895:"Human PubMed Reference:"
1240:(alpha-ketoglutarate) to
1171:
1166:
1162:
1155:
1139:
1120:
1093:
1040:
1029:
1002:
949:
938:
925:
921:
906:
902:
893:
880:
876:
861:
857:
848:
835:
831:
816:
812:
803:
788:
781:
777:
761:
571:
567:
555:
550:
541:
528:
477:
468:
415:
406:
376:
368:
364:
347:
334:
297:
276:
267:
263:
246:
233:
196:
173:
164:
160:
115:
112:
102:
95:
90:
71:
66:
49:
44:
39:
35:
26:
21:
3083:Electron transport chain
2508:10.1074/jbc.273.37.24158
753:lysine catabolic process
748:tricarboxylic acid cycle
3073:P450-containing systems
2134:The Biochemical Journal
1305:Interactive pathway map
1126:Chr 7: 44.61 – 44.71 Mb
723:hippocampus development
642:mitochondrial membranes
596:oxidoreductase activity
481:myocardium of ventricle
3156:Mitochondrial proteins
3078:Cytochrome b6f complex
2823:Pyruvate dehydrogenase
2735:Aldehyde dehydrogenase
2580:10.1074/jbc.M410315200
2249:10.1074/jbc.M409064200
2008:10.1006/geno.1994.1178
1957:10.1073/pnas.89.5.1963
1790:
1394:
1133:Chr 11: 6.24 – 6.31 Mb
703:NADH metabolic process
439:triceps brachii muscle
2918:multienzyme complexes
1789:
1393:
1285:Clinical significance
743:histone succinylation
509:epithelium of stomach
292:Chromosome 11 (mouse)
2538:10.1002/ajmg.a.31641
2365:10.1101/gr.8.11.1097
1796:|alt=TCACycle_WP78
1279:cellular respiration
728:striatum development
688:thalamus development
647:mitochondrial matrix
497:brown adipose tissue
423:gastrocnemius muscle
190:Chromosome 7 (human)
67:List of PDB id codes
40:Available structures
3130:Tryptophan synthase
3120:Polyketide synthase
2864:iron–sulfur protein
2288:10.1038/nature04209
2280:2005Natur.437.1173R
1948:1992PNAS...89.1963K
455:right adrenal gland
2602:, which is in the
2464:10.1101/gr.4039406
2400:10.1101/gr.6.9.791
1791:
1395:
1295:metabolic acidosis
883:ENSMUSG00000020456
678:glycolytic process
671:Biological process
630:Cellular component
579:Molecular function
3138:
3137:
2880:
2879:
2872:Pyruvate synthase
2671:
2670:
2195:10.1002/hep.21828
2146:10.1042/bj1610569
2114:978-0-470-23396-2
1275:Gibbs free energy
1271:citric acid cycle
1250:citric acid cycle
1182:
1181:
1178:
1177:
1151:
1150:
1116:
1115:
1063:
1062:
1025:
1024:
972:
971:
934:
933:
915:
914:
889:
888:
870:
869:
844:
843:
825:
824:
773:
772:
621:chaperone binding
591:metal ion binding
563:
562:
559:
558:
537:
536:
524:
523:
462:
461:
360:
359:
259:
258:
86:
85:
82:
81:
50:Ortholog search:
3163:
2907:
2900:
2893:
2884:
2796:Aldehyde oxidase
2698:
2691:
2684:
2675:
2657:
2637:
2630:
2623:
2614:
2592:
2582:
2557:
2520:
2510:
2501:(37): 24158–64.
2485:
2475:
2441:
2412:
2402:
2377:
2367:
2358:(11): 1097–108.
2342:
2332:
2307:
2274:(7062): 1173–8.
2261:
2251:
2242:(12): 10888–96.
2217:
2216:
2206:
2174:
2168:
2167:
2157:
2125:
2119:
2118:
2100:
2094:
2093:
2083:
2051:
2042:
2041:
2034:
2021:
2020:
2010:
1986:
1980:
1979:
1969:
1959:
1927:
1921:
1920:
1909:
1903:
1902:
1891:
1885:
1875:
1864:
1854:
1832:
1831:
1823:
1716:
1711:
1706:
1701:
1696:
1691:
1686:
1681:
1676:
1671:
1666:
1661:
1656:
1651:
1646:
1641:
1636:
1631:
1626:
1621:
1616:
1611:
1606:
1601:
1596:
1591:
1586:
1581:
1576:
1571:
1566:
1561:
1556:
1551:
1546:
1541:
1536:
1531:
1526:
1521:
1516:
1511:
1506:
1501:
1496:
1491:
1486:
1481:
1476:
1471:
1466:
1461:
1456:
1451:
1446:
1441:
1436:
1431:
1426:
1421:
1416:
1411:
1406:
1401:
1164:
1163:
1135:
1128:
1111:
1067:
1058:
1034:
1030:RefSeq (protein)
1020:
976:
967:
943:
919:
900:
874:
855:
829:
810:
779:
569:
548:
533:
513:digastric muscle
501:plantaris muscle
473:
471:Top expressed in
466:
411:
409:Top expressed in
404:
383:
366:
356:
343:
332:
317:
310:
304:
293:
281:
265:
255:
242:
231:
216:
209:
203:
192:
178:
162:
156:
154:OGDH - orthologs
107:
100:
77:
64:
58:
37:
36:
31:
19:
3171:
3170:
3166:
3165:
3164:
3162:
3161:
3160:
3141:
3140:
3139:
3134:
3039:
2955:
2920:
2911:
2881:
2876:
2854:
2800:
2778:
2756:
2713:
2706:oxidoreductases
2702:
2672:
2667:
2664:
2658:
2644:
2641:
2610:
2595:
2573:(51): 53782–8.
2560:
2523:
2488:
2452:Genome Research
2444:
2415:
2387:Genome Research
2380:
2352:Genome Research
2345:
2310:
2264:
2229:
2225:
2223:Further reading
2220:
2176:
2175:
2171:
2127:
2126:
2122:
2115:
2102:
2101:
2097:
2053:
2052:
2045:
2036:
2035:
2024:
1988:
1987:
1983:
1929:
1928:
1924:
1911:
1910:
1906:
1893:
1892:
1888:
1876:
1867:
1855:
1846:
1842:
1837:
1836:
1835:
1828:"TCACycle_WP78"
1826:
1824:
1820:
1815:
1814:
1813:
1809:TCACycle_WP78
1808:
1803:
1802:
1801:
1795:
1794:
1793:
1792:
1788:
1718:
1717:
1714:
1712:
1709:
1707:
1704:
1702:
1699:
1697:
1694:
1692:
1689:
1687:
1684:
1682:
1679:
1677:
1674:
1672:
1669:
1667:
1664:
1662:
1659:
1657:
1654:
1652:
1649:
1647:
1644:
1642:
1639:
1637:
1634:
1632:
1629:
1627:
1624:
1622:
1619:
1617:
1614:
1612:
1609:
1607:
1604:
1602:
1599:
1597:
1594:
1592:
1589:
1587:
1584:
1582:
1579:
1577:
1574:
1572:
1569:
1567:
1564:
1562:
1559:
1557:
1554:
1552:
1549:
1547:
1544:
1542:
1539:
1537:
1534:
1532:
1529:
1527:
1524:
1522:
1519:
1517:
1514:
1512:
1509:
1507:
1504:
1502:
1499:
1497:
1494:
1492:
1489:
1487:
1484:
1482:
1479:
1477:
1474:
1472:
1469:
1467:
1464:
1462:
1459:
1457:
1454:
1452:
1449:
1447:
1444:
1442:
1439:
1437:
1434:
1432:
1429:
1427:
1424:
1422:
1419:
1417:
1414:
1412:
1409:
1407:
1404:
1402:
1399:
1396:
1392:
1307:
1299:hyperlactatemia
1287:
1267:
1258:
1247:
1230:
1221:
1213:
1208:
1173:View/Edit Mouse
1168:View/Edit Human
1131:
1124:
1121:Location (UCSC)
1107:
1103:
1099:
1095:
1089:
1085:
1081:
1077:
1073:
1054:
1050:
1046:
1042:
1016:
1012:
1008:
1004:
998:
994:
990:
986:
982:
963:
959:
955:
951:
864:ENSG00000105953
757:
666:
625:
529:
520:
515:
511:
507:
503:
499:
495:
493:muscle of thigh
491:
489:right ventricle
487:
483:
469:
458:
453:
449:
445:
441:
437:
433:
429:
427:muscle of thigh
425:
421:
407:
351:
338:
330:
320:
319:
318:
311:
291:
268:Gene location (
250:
237:
229:
219:
218:
217:
210:
188:
165:Gene location (
116:
103:
96:
73:
51:
17:
12:
11:
5:
3169:
3167:
3159:
3158:
3153:
3143:
3142:
3136:
3135:
3133:
3132:
3127:
3122:
3117:
3112:
3111:
3110:
3105:
3095:
3090:
3085:
3080:
3075:
3070:
3069:
3068:
3066:Dihydroorotase
3063:
3058:
3047:
3045:
3041:
3040:
3038:
3037:
3036:
3035:
3030:
3025:
3020:
3010:
3009:
3008:
3003:
2998:
2988:
2987:
2986:
2981:
2976:
2965:
2963:
2957:
2956:
2954:
2953:
2952:
2951:
2946:
2936:
2930:
2928:
2926:Photosynthesis
2922:
2921:
2912:
2910:
2909:
2902:
2895:
2887:
2878:
2877:
2875:
2874:
2868:
2866:
2856:
2855:
2853:
2852:
2851:
2850:
2845:
2840:
2835:
2825:
2820:
2814:
2812:
2802:
2801:
2799:
2798:
2792:
2790:
2780:
2779:
2777:
2776:
2770:
2768:
2758:
2757:
2755:
2754:
2749:
2748:
2747:
2742:
2731:
2729:
2715:
2714:
2703:
2701:
2700:
2693:
2686:
2678:
2669:
2668:
2666:
2665:
2659:
2652:
2649:
2646:
2645:
2642:
2640:
2639:
2632:
2625:
2617:
2594:
2593:
2558:
2521:
2486:
2442:
2413:
2393:(9): 791–806.
2378:
2343:
2323:(16): 8971–4.
2308:
2262:
2226:
2224:
2221:
2219:
2218:
2189:(5): 1436–42.
2169:
2120:
2113:
2095:
2043:
2022:
1981:
1922:
1904:
1886:
1865:
1843:
1841:
1838:
1834:
1833:
1817:
1816:
1805:
1804:
1719:
1713:
1708:
1703:
1698:
1693:
1688:
1683:
1678:
1673:
1668:
1663:
1658:
1653:
1648:
1643:
1638:
1633:
1628:
1623:
1618:
1613:
1608:
1603:
1598:
1593:
1588:
1583:
1578:
1573:
1568:
1563:
1558:
1553:
1548:
1543:
1538:
1533:
1528:
1523:
1518:
1513:
1508:
1503:
1498:
1493:
1488:
1483:
1478:
1473:
1468:
1463:
1458:
1453:
1448:
1443:
1438:
1433:
1428:
1423:
1418:
1413:
1408:
1403:
1398:
1397:
1323:
1322:
1321:
1320:
1318:
1317:
1316:
1315:
1314:
1313:
1306:
1303:
1286:
1283:
1266:
1263:
1257:
1254:
1245:
1238:2-oxoglutarate
1229:
1226:
1220:
1217:
1212:
1209:
1207:
1204:
1187:also known as
1180:
1179:
1176:
1175:
1170:
1160:
1159:
1153:
1152:
1149:
1148:
1146:
1144:
1137:
1136:
1129:
1122:
1118:
1117:
1114:
1113:
1091:
1090:
1064:
1061:
1060:
1038:
1037:
1031:
1027:
1026:
1023:
1022:
1000:
999:
973:
970:
969:
947:
946:
940:
936:
935:
932:
931:
923:
922:
916:
913:
912:
904:
903:
897:
891:
890:
887:
886:
878:
877:
871:
868:
867:
859:
858:
852:
846:
845:
842:
841:
833:
832:
826:
823:
822:
814:
813:
807:
801:
800:
795:
790:
786:
785:
775:
774:
771:
770:
759:
758:
756:
755:
750:
745:
740:
735:
730:
725:
720:
715:
710:
705:
700:
695:
690:
685:
680:
674:
672:
668:
667:
665:
664:
659:
654:
649:
644:
639:
633:
631:
627:
626:
624:
623:
618:
613:
608:
603:
598:
593:
588:
582:
580:
576:
575:
565:
564:
561:
560:
557:
556:
553:
552:
545:
539:
538:
535:
534:
526:
525:
522:
521:
519:
518:
514:
510:
506:
502:
498:
494:
490:
486:
482:
478:
475:
474:
463:
460:
459:
457:
456:
452:
448:
444:
440:
436:
432:
431:left ventricle
428:
424:
420:
416:
413:
412:
400:
399:
391:
380:
374:
373:
370:RNA expression
362:
361:
358:
357:
349:
345:
344:
336:
333:
328:
322:
321:
312:
305:
299:
295:
294:
289:
283:
282:
274:
273:
261:
260:
257:
256:
248:
244:
243:
235:
232:
227:
221:
220:
211:
204:
198:
194:
193:
186:
180:
179:
171:
170:
158:
157:
114:
110:
109:
101:
93:
92:
88:
87:
84:
83:
80:
79:
69:
68:
60:
59:
48:
42:
41:
33:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
3168:
3157:
3154:
3152:
3149:
3148:
3146:
3131:
3128:
3126:
3123:
3121:
3118:
3116:
3113:
3109:
3106:
3104:
3101:
3100:
3099:
3096:
3094:
3091:
3089:
3086:
3084:
3081:
3079:
3076:
3074:
3071:
3067:
3064:
3062:
3059:
3057:
3054:
3053:
3052:
3049:
3048:
3046:
3042:
3034:
3031:
3029:
3026:
3024:
3021:
3019:
3016:
3015:
3014:
3011:
3007:
3004:
3002:
2999:
2997:
2994:
2993:
2992:
2989:
2985:
2982:
2980:
2977:
2975:
2972:
2971:
2970:
2967:
2966:
2964:
2962:
2961:Dehydrogenase
2958:
2950:
2947:
2945:
2942:
2941:
2940:
2937:
2935:
2932:
2931:
2929:
2927:
2923:
2919:
2915:
2908:
2903:
2901:
2896:
2894:
2889:
2888:
2885:
2873:
2870:
2869:
2867:
2865:
2861:
2857:
2849:
2846:
2844:
2841:
2839:
2836:
2834:
2831:
2830:
2829:
2826:
2824:
2821:
2819:
2816:
2815:
2813:
2811:
2807:
2803:
2797:
2794:
2793:
2791:
2789:
2785:
2781:
2775:
2772:
2771:
2769:
2767:
2763:
2759:
2753:
2750:
2746:
2743:
2741:
2738:
2737:
2736:
2733:
2732:
2730:
2728:
2724:
2720:
2716:
2711:
2707:
2704:Aldehyde/oxo
2699:
2694:
2692:
2687:
2685:
2680:
2679:
2676:
2662:
2656:
2651:
2647:
2638:
2633:
2631:
2626:
2624:
2619:
2618:
2615:
2611:
2608:
2607:
2605:
2604:public domain
2601:
2590:
2586:
2581:
2576:
2572:
2568:
2564:
2559:
2555:
2551:
2547:
2543:
2539:
2535:
2531:
2527:
2522:
2518:
2514:
2509:
2504:
2500:
2496:
2492:
2487:
2483:
2479:
2474:
2469:
2465:
2461:
2457:
2453:
2449:
2443:
2439:
2435:
2431:
2427:
2423:
2419:
2414:
2410:
2406:
2401:
2396:
2392:
2388:
2384:
2379:
2375:
2371:
2366:
2361:
2357:
2353:
2349:
2344:
2340:
2336:
2331:
2326:
2322:
2318:
2314:
2309:
2305:
2301:
2297:
2293:
2289:
2285:
2281:
2277:
2273:
2269:
2263:
2259:
2255:
2250:
2245:
2241:
2237:
2233:
2228:
2227:
2222:
2214:
2210:
2205:
2200:
2196:
2192:
2188:
2184:
2180:
2173:
2170:
2165:
2161:
2156:
2151:
2147:
2143:
2140:(3): 569–81.
2139:
2135:
2131:
2124:
2121:
2116:
2110:
2106:
2099:
2096:
2091:
2087:
2082:
2077:
2073:
2069:
2066:(3): 639–51.
2065:
2061:
2057:
2050:
2048:
2044:
2039:
2033:
2031:
2029:
2027:
2023:
2018:
2014:
2009:
2004:
2000:
1996:
1992:
1985:
1982:
1977:
1973:
1968:
1963:
1958:
1953:
1949:
1945:
1942:(5): 1963–7.
1941:
1937:
1933:
1926:
1923:
1918:
1914:
1908:
1905:
1900:
1896:
1890:
1887:
1883:
1879:
1874:
1872:
1870:
1866:
1862:
1858:
1853:
1851:
1849:
1845:
1839:
1829:
1822:
1819:
1812:
1807:
1799:
1723:
1327:
1312:
1311:
1304:
1302:
1300:
1296:
1292:
1284:
1282:
1280:
1276:
1272:
1264:
1262:
1255:
1253:
1251:
1243:
1239:
1235:
1227:
1225:
1218:
1216:
1210:
1205:
1203:
1201:
1198:
1194:
1190:
1186:
1174:
1169:
1165:
1161:
1158:
1154:
1147:
1145:
1142:
1138:
1134:
1130:
1127:
1123:
1119:
1112:
1110:
1106:
1102:
1098:
1092:
1088:
1084:
1080:
1076:
1072:
1068:
1065:
1059:
1057:
1053:
1049:
1045:
1039:
1035:
1032:
1028:
1021:
1019:
1015:
1011:
1007:
1001:
997:
993:
989:
985:
981:
977:
974:
968:
966:
962:
958:
954:
948:
944:
941:
939:RefSeq (mRNA)
937:
930:
929:
924:
920:
917:
911:
910:
905:
901:
898:
896:
892:
885:
884:
879:
875:
872:
866:
865:
860:
856:
853:
851:
847:
840:
839:
834:
830:
827:
821:
820:
815:
811:
808:
806:
802:
799:
796:
794:
791:
787:
784:
780:
776:
769:
765:
760:
754:
751:
749:
746:
744:
741:
739:
736:
734:
731:
729:
726:
724:
721:
719:
716:
714:
711:
709:
706:
704:
701:
699:
696:
694:
691:
689:
686:
684:
681:
679:
676:
675:
673:
670:
669:
663:
660:
658:
655:
653:
652:mitochondrion
650:
648:
645:
643:
640:
638:
635:
634:
632:
629:
628:
622:
619:
617:
614:
612:
609:
607:
604:
602:
599:
597:
594:
592:
589:
587:
584:
583:
581:
578:
577:
574:
573:Gene ontology
570:
566:
554:
549:
546:
544:
540:
532:
527:
517:soleus muscle
516:
512:
508:
504:
500:
496:
492:
488:
484:
480:
479:
476:
472:
467:
464:
454:
450:
446:
442:
438:
435:right auricle
434:
430:
426:
422:
419:apex of heart
418:
417:
414:
410:
405:
402:
401:
398:
396:
392:
390:
389:
385:
384:
381:
379:
375:
371:
367:
363:
355:
350:
346:
342:
337:
331:11|11 A1
327:
323:
316:
309:
303:
296:
288:
284:
280:
275:
271:
266:
262:
254:
249:
245:
241:
236:
226:
222:
215:
208:
202:
195:
191:
185:
181:
177:
172:
168:
163:
159:
155:
151:
147:
143:
139:
135:
131:
127:
123:
119:
111:
106:
99:
94:
89:
78:
76:
70:
65:
62:
61:
57:
54:
47:
43:
38:
34:
30:
25:
20:
2995:
2660:
2609:
2597:
2596:
2570:
2566:
2532:(7): 763–7.
2529:
2525:
2498:
2494:
2458:(1): 55–65.
2455:
2451:
2424:(2): 261–6.
2421:
2417:
2390:
2386:
2355:
2351:
2320:
2316:
2271:
2267:
2239:
2235:
2186:
2182:
2172:
2137:
2133:
2123:
2104:
2098:
2063:
2059:
2001:(2): 324–6.
1998:
1994:
1984:
1939:
1935:
1925:
1916:
1907:
1898:
1889:
1821:
1309:
1308:
1288:
1268:
1259:
1242:succinyl-CoA
1231:
1222:
1214:
1196:
1188:
1184:
1183:
1109:NP_001348834
1105:NP_001348833
1101:NP_001348832
1097:NP_001348831
1094:
1083:NP_001239217
1079:NP_001239216
1075:NP_001239212
1071:NP_001239211
1056:NP_001350452
1048:NP_001158508
1044:NP_001003941
1041:
1018:NM_001361905
1014:NM_001361904
1010:NM_001361903
1006:NM_001361902
1003:
992:NM_001252288
988:NM_001252287
984:NM_001252283
980:NM_001252282
965:NM_001363523
957:NM_001165036
953:NM_001003941
950:
926:
907:
881:
862:
836:
817:
797:
792:
393:
386:
113:External IDs
72:
2939:Photosystem
2643:PDB gallery
1248:during the
251:44,709,066
238:44,606,572
91:Identifiers
3145:Categories
2766:cytochrome
2183:Hepatology
1884:, May 2017
1863:, May 2017
1840:References
1265:Regulation
718:metabolism
397:(ortholog)
352:6,306,642
339:6,241,633
134:HomoloGene
2810:disulfide
1291:hypotonia
1256:Mechanism
1206:Structure
1087:NP_035086
1052:NP_002532
996:NM_010956
961:NM_002541
783:Orthologs
142:GeneCards
2589:15466852
2554:11529600
2546:17343268
2482:16344560
2296:16189514
2258:15649899
2213:17657817
2090:17367808
1995:Genomics
1880:–
1859:–
1228:Function
1157:Wikidata
762:Sources:
2914:Enzymes
2517:9727038
2473:1356129
2438:7622061
2409:8889548
2374:9847074
2339:2188967
2304:4427026
2276:Bibcode
2204:3731127
2155:1164543
2081:7611002
2017:8020988
1976:1542694
1944:Bibcode
1882:Ensembl
1861:Ensembl
1319:[[File:
1219:Protein
895:UniProt
850:Ensembl
789:Species
768:QuickGO
662:nucleus
637:cytosol
372:pattern
130:1098267
98:Aliases
3023:BCKDHB
3018:BCKDHA
2838:BCKDHB
2833:BCKDHA
2788:oxygen
2587:
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2015:
1974:
1964:
1297:, and
1244:and CO
1193:enzyme
1191:is an
1143:search
1141:PubMed
928:Q60597
909:Q02218
805:Entrez
543:BioGPS
447:glutes
122:613022
3108:HADHB
3103:HADHA
3044:Other
2860:1.2.7
2806:1.2.4
2784:1.2.3
2762:1.2.2
2719:1.2.1
2550:S2CID
2300:S2CID
1967:48574
838:18293
798:Mouse
793:Human
764:Amigo
395:Mouse
388:Human
335:Start
270:Mouse
234:Start
167:Human
138:55662
3001:DLST
2996:OGDH
2727:NADP
2712:1.2)
2661:3ery
2585:PMID
2542:PMID
2530:143A
2513:PMID
2478:PMID
2434:PMID
2418:Gene
2405:PMID
2370:PMID
2335:PMID
2292:PMID
2254:PMID
2209:PMID
2160:PMID
2109:ISBN
2086:PMID
2013:PMID
1972:PMID
1811:edit
1798:edit
1211:Gene
1200:gene
1197:OGDH
819:4967
378:Bgee
326:Band
287:Chr.
230:7p13
225:Band
184:Chr.
146:OGDH
118:OMIM
105:OGDH
75:3ERY
56:RCSB
53:PDBe
22:OGDH
3051:CAD
3033:DLD
3028:DBT
3006:DLD
2848:DLD
2843:DBT
2725:or
2723:NAD
2575:doi
2571:279
2534:doi
2503:doi
2499:273
2468:PMC
2460:doi
2426:doi
2422:159
2395:doi
2360:doi
2325:doi
2321:265
2284:doi
2272:437
2244:doi
2240:280
2199:PMC
2191:doi
2150:PMC
2142:doi
2138:161
2076:PMC
2068:doi
2064:368
2003:doi
1962:PMC
1952:doi
551:n/a
348:End
247:End
150:OMA
126:MGI
46:PDB
3147::
2984:E3
2979:E2
2974:E1
2949:II
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2862::
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