2042:
2012:
2027:
318:
1997:
295:
1952:
1937:
1967:
192:
217:
1982:
583:
576:
569:
324:
223:
29:
2537:
1552:"Maple syrup urine disease caused by a partial deletion in the inner E2 core domain of the branched chain alpha-keto acid dehydrogenase complex due to aberrant splicing. A single base deletion at a 5'-splice donor site of an intron of the E2 gene disrupts the consensus sequence in this region"
1657:
Lau KS, Griffin TA, Hu CW, Chuang DT (1988). "Conservation of primary structure in the lipoyl-bearing and dihydrolipoyl dehydrogenase binding domains of mammalian branched-chain alpha-keto acid dehydrogenase complex: molecular cloning of human and bovine transacylase (E2) cDNAs".
2041:
2011:
55:
2026:
1996:
1420:
Zneimer SM, Lau KS, Eddy RL, et al. (1991). "Regional assignment of two genes of the human branched-chain alpha-keto acid dehydrogenase complex: the E1 beta gene (BCKDHB) to chromosome 6p21-22 and the E2 gene (DBT) to chromosome 1p31".
1450:
Chuang DT, Fisher CW, Lau KS, et al. (1991). "Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex".
1951:
1936:
1829:
Wynn RM, Kochi H, Cox RP, Chuang DT (1994). "Differential processing of human and rat E1 alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence".
1390:
Fisher CW, Lau KS, Fisher CR, et al. (1991). "A 17-bp insertion and a Phe215----Cys missense mutation in the dihydrolipoyl transacylase (E2) mRNA from a thiamine-responsive maple syrup urine disease patient WG-34".
1966:
1861:"Occurrence of a 2-bp (AT) deletion allele and a nonsense (G-to-T) mutant allele at the E2 (DBT) locus of six patients with maple syrup urine disease: multiple-exon skipping as a secondary effect of the mutations"
1214:
Lau KS, Chuang JL, Herring WJ, Danner DJ, Cox RP, Chuang DT (Dec 1992). "The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain alpha-keto acid dehydrogenase complex".
2050:: Crystal structure of a cubic core of the dihydrolipoamide acyltransferase (E2b) component in the branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), Isobutyryl-Coenzyme A-bound form
2020:: Crystal structure of a cubic core of the dihydrolipoamide acyltransferase (E2b) component in the branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), Oxidized Coenzyme A-bound form
1628:
Nobukuni Y, Mitsubuchi H, Endo F, Matsuda I (1989). "Complete primary structure of the transacylase (E2b) subunit of the human branched chain alpha-keto acid dehydrogenase complex".
2082:
2035:: Crystal structure of a cubic core of the dihydrolipoamide acyltransferase (E2b) component in the branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), Coenzyme A-bound form
1981:
1474:"Molecular genetic basis of maple syrup urine disease in a family with two defective alleles for branched chain acyltransferase and localization of the gene to human chromosome 1"
1251:"Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene"
2399:
2214:
1076:
2005:: Crystal structure of a cubic core of the dihydrolipoamide acyltransferase (E2b) component in the branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), apo form
331:
230:
2291:
2138:
1509:"Premature termination of transcription and alternative splicing in the human transacylase (E2) gene of the branched-chain alpha-ketoacid dehydrogenase complex"
2113:
1788:"Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects"
2075:
791:
772:
153:
1687:
Litwer S, Danner DJ (1985). "Identification of a cDNA clone in lambda gt11 for the transacylase component of branched chain ketoacid dehydrogenase".
2606:
1960:: Solution Structure of the Lipoic Acid-Bearing Domain of the E2 component of Human, Mitochondrial Branched-Chain alpha-Ketoacid Dehydrogenase
1945:: Solution Structure of the Lipoic Acid-Bearing Domain of the E2 component of Human, Mitochondrial Branched-Chain alpha-Ketoacid Dehydrogenase
2582:
2068:
1916:
1196:
1178:
2601:
1357:"Branched-chain alpha-ketoacid dehydrogenase kinase. Molecular cloning, expression, and sequence similarity with histidine protein kinases"
2484:
2284:
999:
317:
2377:
2204:
2160:
1006:
2131:
2109:
294:
1975:: Solution Structure of the subunit binding domain (hbSBD) of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase
1293:
2611:
2442:
1165:
1144:
2447:
2419:
2392:
2370:
2355:
2320:
2277:
2234:
1595:"Construction and nucleotide sequence of a cDNA encoding the full-length preprotein for human branched chain acyltransferase"
1161:
216:
191:
52:
1140:
2365:
1080:
133:
2126:
330:
229:
2575:
2246:
2192:
2170:
2148:
2105:
1909:
1753:"Nucleotide sequence of a cDNA for branched chain acyltransferase with analysis of the deduced protein structure"
1718:"Mitochondrial import and processing of an in vitro synthesized human prebranched chain acyltransferase fragment"
1112:
323:
222:
2469:
2096:
836:
141:
2250:
2501:
2479:
2459:
817:
2464:
2360:
2209:
2121:
1059:
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
124:, BCATE2, BCKAD-E2, BCKADE2, E2, E2B, BCOADC-E2, dihydrolipoamide branched chain transacylase E2, BCKDH-E2
2568:
1902:
1520:
205:
120:
2516:
2506:
2474:
1116:
982:
931:
2511:
1343:
165:
978:
957:
927:
906:
2258:
1882:
1847:
1817:
1774:
1739:
1704:
1675:
1645:
1616:
1581:
1538:
1495:
1460:
1438:
1408:
1378:
1335:
1272:
1231:
113:
45:
2552:
2182:
1990:: Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase
1872:
1839:
1807:
1799:
1764:
1729:
1696:
1667:
1637:
1606:
1571:
1563:
1528:
1485:
1430:
1400:
1368:
1325:
1262:
1223:
410:
341:
285:
240:
582:
575:
568:
2304:
385:
161:
2060:
1524:
1314:"Mammalian alpha-keto acid dehydrogenase complexes: gene regulation and genetic defects"
2452:
2335:
2312:
2092:
1877:
1860:
1812:
1787:
1734:
1717:
1490:
1473:
1769:
1752:
1611:
1594:
1576:
1551:
1373:
1356:
1267:
1250:
706:
701:
685:
680:
675:
670:
665:
649:
644:
639:
634:
629:
624:
2595:
2347:
2330:
1843:
1700:
1641:
1533:
1508:
1434:
1404:
1227:
1104:
1100:
611:
1347:
28:
2548:
1096:
403:
182:
145:
1249:
Lau KS, Herring WJ, Chuang JL, McKean M, Danner DJ, Cox RP, Chuang DT (Dec 1992).
2269:
169:
2325:
1330:
1313:
1201:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1183:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1107:(E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit.
486:
2437:
2152:
1084:
302:
199:
149:
2196:
1119:
have been described, but their biological validity has not been determined.
1108:
736:
546:
424:
369:
356:
268:
255:
157:
1083:
enzyme complex involved in the breakdown of the branched-chain amino acids
1886:
1851:
1821:
1778:
1743:
1708:
1679:
1649:
1620:
1585:
1542:
1499:
1464:
1442:
1412:
1382:
1339:
1276:
1235:
1046:
1041:
1030:
881:
862:
1671:
1088:
848:
803:
1803:
1567:
90:
86:
82:
78:
74:
2409:
2404:
2300:
2224:
2219:
2174:
1092:
1062:
1014:
758:
2536:
640:
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity
2494:
2489:
1294:"Entrez Gene: DBT dihydrolipoamide branched chain transacylase E2"
721:
717:
2544:
2387:
2382:
1069:
137:
2273:
2064:
1898:
591:
1894:
1095:. The BCKD complex is thought to be composed of a core of 24
1550:
Mitsubuchi H, Nobukuni Y, Akaboshi I, et al. (1991).
393:
2556:
671:
mitochondrial alpha-ketoglutarate dehydrogenase complex
1751:
Hummel KB, Litwer S, Bradford AP, et al. (1988).
1859:
Fisher CW, Fisher CR, Chuang JL, et al. (1993).
558:
2400:
Branched-chain alpha-keto acid dehydrogenase complex
2215:
Branched-chain alpha-keto acid dehydrogenase complex
1593:
Danner DJ, Litwer S, Herring WJ, Pruckler J (1989).
1077:
branched-chain alpha-keto acid dehydrogenase complex
2502:
Phosphoenolpyruvate sugar phosphotransferase system
2430:
2346:
2311:
2245:
2191:
2169:
2147:
2104:
1355:Popov KM, Zhao Y, Shimomura Y, et al. (1992).
971:
950:
920:
899:
1472:Herring WJ, Litwer S, Weber JL, Danner DJ (1991).
1157:
1155:
1153:
1136:
1134:
1132:
340:
239:
2321:Photosynthetic reaction center complex proteins
2139:Mycothiol-dependent formaldehyde dehydrogenase
1507:Lau KS, Lee J, Fisher CW, et al. (1991).
1162:GRCm38: Ensembl release 89: ENSMUSG00000000340
2576:
2285:
2076:
1910:
8:
1141:GRCh38: Ensembl release 89: ENSG00000137992
707:branched-chain amino acid catabolic process
2583:
2569:
2292:
2278:
2270:
2083:
2069:
2061:
1917:
1903:
1895:
732:
607:
381:
280:
177:
63:
1876:
1811:
1768:
1733:
1610:
1575:
1532:
1489:
1372:
1329:
1266:
1288:
1286:
1932:
1128:
16:Mammalian protein found in Homo sapiens
18:
345:
306:
301:
244:
203:
198:
7:
2533:
2531:
2485:Mitochondrial trifunctional protein
2555:. You can help Knowledge (XXG) by
2205:Oxoglutarate dehydrogenase complex
2161:Formate dehydrogenase (cytochrome)
1786:Chuang DT, Niu WL, Cox RP (1982).
968:
947:
917:
896:
872:
853:
827:
808:
782:
763:
563:
481:
419:
398:
14:
2132:Long-chain-aldehyde dehydrogenase
1065:that in humans is encoded by the
2535:
2040:
2025:
2010:
1995:
1980:
1965:
1950:
1935:
1115:, type 2. Alternatively spliced
630:ubiquitin protein ligase binding
581:
574:
567:
471:inferior ganglion of vagus nerve
329:
322:
316:
293:
228:
221:
215:
190:
27:
2443:Carbamoyl phosphate synthase II
2448:Aspartate carbamoyltransferase
2356:Pyruvate dehydrogenase complex
1099:(E2) subunits, and associated
592:More reference expression data
547:More reference expression data
1:
2607:Human chromosome 1 gene stubs
2480:Glycine decarboxylase complex
2475:Fatty acid synthetase complex
1770:10.1016/S0021-9258(18)68766-6
1689:Biochem. Biophys. Res. Commun
1630:Biochem. Biophys. Res. Commun
1612:10.1016/S0021-9258(18)83297-5
1393:Biochem. Biophys. Res. Commun
1374:10.1016/S0021-9258(18)42179-5
1268:10.1016/S0021-9258(18)35950-7
314:
213:
1844:10.1016/0304-4165(94)90161-9
1716:Litwer S, Danner DJ (1988).
1701:10.1016/0006-291X(85)91333-6
1642:10.1016/0006-291X(89)91347-8
1534:10.1016/0014-5793(91)80155-V
1435:10.1016/0888-7543(91)90458-Q
1405:10.1016/0006-291X(91)91489-Y
1312:Patel MS, Harris RA (1995).
1228:10.1016/0167-4781(92)90169-z
2602:Genes on human chromosome 1
1331:10.1096/fasebj.9.12.7672509
2628:
2530:
2512:Sucrase-isomaltase complex
2378:Oxoglutarate dehydrogenase
2127:Acetaldehyde dehydrogenase
645:acetyltransferase activity
521:retinal pigment epithelium
1930:
1197:"Mouse PubMed Reference:"
1179:"Human PubMed Reference:"
1113:maple syrup urine disease
1045:
1040:
1036:
1029:
1013:
1007:Chr 3: 116.31 – 116.34 Mb
1000:Chr 1: 100.19 – 100.25 Mb
994:
975:
954:
943:
924:
903:
892:
879:
875:
860:
856:
847:
834:
830:
815:
811:
802:
789:
785:
770:
766:
757:
742:
735:
731:
715:
610:
606:
589:
566:
557:
544:
493:
484:
431:
422:
392:
384:
380:
363:
350:
313:
292:
283:
279:
262:
249:
212:
189:
180:
176:
131:
128:
118:
111:
106:
71:
66:
49:
44:
39:
35:
26:
21:
2470:Electron transport chain
625:acyltransferase activity
467:external globus pallidus
2460:P450-containing systems
1111:in this gene result in
533:myocardium of ventricle
2612:Mitochondrial proteins
2465:Cytochrome b6f complex
2210:Pyruvate dehydrogenase
2122:Aldehyde dehydrogenase
1832:Biochim. Biophys. Acta
676:mitochondrial nucleoid
451:ventral tegmental area
2305:multienzyme complexes
517:epithelium of stomach
1216:Biochim Biophys Acta
666:mitochondrial matrix
635:transferase activity
497:brown adipose tissue
347:3 G1|3 50.37 cM
308:Chromosome 3 (mouse)
206:Chromosome 1 (human)
67:List of PDB id codes
40:Available structures
2517:Tryptophan synthase
2507:Polyketide synthase
2251:iron–sulfur protein
1672:10.1021/bi00406a025
1525:1991FEBSL.279..229L
1117:transcript variants
1081:inner-mitochondrial
650:lipoic acid binding
463:trigeminal ganglion
2543:This article on a
837:ENSMUSG00000000340
695:Biological process
659:Cellular component
618:Molecular function
509:left lobe of liver
505:intercostal muscle
435:buccal mucosa cell
2564:
2563:
2525:
2524:
2267:
2266:
2259:Pyruvate synthase
2058:
2057:
1865:Am. J. Hum. Genet
1804:10.1042/bj2000059
1722:Am. J. Hum. Genet
1568:10.1172/JCI115120
1478:Am. J. Hum. Genet
1056:
1055:
1052:
1051:
1025:
1024:
990:
989:
965:
964:
939:
938:
914:
913:
888:
887:
869:
868:
843:
842:
824:
823:
798:
797:
779:
778:
727:
726:
602:
601:
598:
597:
553:
552:
540:
539:
478:
477:
376:
375:
275:
274:
102:
101:
98:
97:
50:Ortholog search:
2619:
2585:
2578:
2571:
2539:
2532:
2294:
2287:
2280:
2271:
2183:Aldehyde oxidase
2085:
2078:
2071:
2062:
2044:
2029:
2014:
1999:
1984:
1969:
1954:
1939:
1919:
1912:
1905:
1896:
1890:
1880:
1855:
1825:
1815:
1782:
1772:
1747:
1737:
1712:
1683:
1653:
1624:
1614:
1589:
1579:
1546:
1536:
1503:
1493:
1468:
1446:
1416:
1386:
1376:
1367:(19): 13127–30.
1351:
1333:
1298:
1297:
1290:
1281:
1280:
1270:
1246:
1240:
1239:
1211:
1205:
1204:
1193:
1187:
1186:
1175:
1169:
1159:
1148:
1138:
1038:
1037:
1009:
1002:
985:
969:
960:
948:
944:RefSeq (protein)
934:
918:
909:
897:
873:
854:
828:
809:
783:
764:
733:
608:
594:
585:
578:
571:
564:
549:
489:
487:Top expressed in
482:
443:endothelial cell
427:
425:Top expressed in
420:
399:
382:
372:
359:
348:
333:
326:
320:
309:
297:
281:
271:
258:
247:
232:
225:
219:
208:
194:
178:
172:
123:
116:
93:
64:
58:
37:
36:
31:
19:
2627:
2626:
2622:
2621:
2620:
2618:
2617:
2616:
2592:
2591:
2590:
2589:
2528:
2526:
2521:
2426:
2342:
2307:
2298:
2268:
2263:
2241:
2187:
2165:
2143:
2100:
2093:oxidoreductases
2089:
2059:
2054:
2051:
2045:
2036:
2030:
2021:
2015:
2006:
2000:
1991:
1985:
1976:
1970:
1961:
1955:
1946:
1940:
1926:
1923:
1893:
1858:
1828:
1785:
1750:
1715:
1686:
1656:
1627:
1592:
1556:J. Clin. Invest
1549:
1506:
1471:
1449:
1419:
1389:
1354:
1324:(12): 1164–72.
1311:
1307:
1305:Further reading
1302:
1301:
1292:
1291:
1284:
1261:(33): 24090–6.
1248:
1247:
1243:
1213:
1212:
1208:
1195:
1194:
1190:
1177:
1176:
1172:
1160:
1151:
1139:
1130:
1125:
1047:View/Edit Mouse
1042:View/Edit Human
1005:
998:
995:Location (UCSC)
981:
977:
956:
930:
926:
905:
818:ENSG00000137992
711:
690:
654:
590:
580:
579:
573:
572:
545:
536:
531:
527:
523:
519:
515:
511:
507:
503:
499:
485:
474:
469:
465:
461:
457:
453:
449:
445:
441:
437:
423:
367:
354:
346:
336:
335:
334:
327:
307:
284:Gene location (
266:
253:
245:
235:
234:
233:
226:
204:
181:Gene location (
170:DBT - orthologs
132:
119:
112:
73:
51:
17:
12:
11:
5:
2625:
2623:
2615:
2614:
2609:
2604:
2594:
2593:
2588:
2587:
2580:
2573:
2565:
2562:
2561:
2540:
2523:
2522:
2520:
2519:
2514:
2509:
2504:
2499:
2498:
2497:
2492:
2482:
2477:
2472:
2467:
2462:
2457:
2456:
2455:
2453:Dihydroorotase
2450:
2445:
2434:
2432:
2428:
2427:
2425:
2424:
2423:
2422:
2417:
2412:
2407:
2397:
2396:
2395:
2390:
2385:
2375:
2374:
2373:
2368:
2363:
2352:
2350:
2344:
2343:
2341:
2340:
2339:
2338:
2333:
2323:
2317:
2315:
2313:Photosynthesis
2309:
2308:
2299:
2297:
2296:
2289:
2282:
2274:
2265:
2264:
2262:
2261:
2255:
2253:
2243:
2242:
2240:
2239:
2238:
2237:
2232:
2227:
2222:
2212:
2207:
2201:
2199:
2189:
2188:
2186:
2185:
2179:
2177:
2167:
2166:
2164:
2163:
2157:
2155:
2145:
2144:
2142:
2141:
2136:
2135:
2134:
2129:
2118:
2116:
2102:
2101:
2090:
2088:
2087:
2080:
2073:
2065:
2056:
2055:
2053:
2052:
2046:
2039:
2037:
2031:
2024:
2022:
2016:
2009:
2007:
2001:
1994:
1992:
1986:
1979:
1977:
1971:
1964:
1962:
1956:
1949:
1947:
1941:
1934:
1931:
1928:
1927:
1924:
1922:
1921:
1914:
1907:
1899:
1892:
1891:
1856:
1826:
1783:
1763:(13): 6165–8.
1748:
1713:
1684:
1666:(6): 1972–81.
1654:
1636:(3): 1035–41.
1625:
1605:(13): 7742–6.
1590:
1562:(4): 1207–11.
1547:
1504:
1469:
1453:Mol. Biol. Med
1447:
1417:
1387:
1352:
1308:
1306:
1303:
1300:
1299:
1282:
1241:
1206:
1188:
1170:
1149:
1127:
1126:
1124:
1121:
1054:
1053:
1050:
1049:
1044:
1034:
1033:
1027:
1026:
1023:
1022:
1020:
1018:
1011:
1010:
1003:
996:
992:
991:
988:
987:
973:
972:
966:
963:
962:
952:
951:
945:
941:
940:
937:
936:
922:
921:
915:
912:
911:
901:
900:
894:
890:
889:
886:
885:
877:
876:
870:
867:
866:
858:
857:
851:
845:
844:
841:
840:
832:
831:
825:
822:
821:
813:
812:
806:
800:
799:
796:
795:
787:
786:
780:
777:
776:
768:
767:
761:
755:
754:
749:
744:
740:
739:
729:
728:
725:
724:
713:
712:
710:
709:
704:
698:
696:
692:
691:
689:
688:
683:
678:
673:
668:
662:
660:
656:
655:
653:
652:
647:
642:
637:
632:
627:
621:
619:
615:
614:
604:
603:
600:
599:
596:
595:
587:
586:
561:
555:
554:
551:
550:
542:
541:
538:
537:
535:
534:
530:
526:
522:
518:
514:
513:lacrimal gland
510:
506:
502:
498:
494:
491:
490:
479:
476:
475:
473:
472:
468:
464:
460:
456:
452:
448:
444:
440:
436:
432:
429:
428:
416:
415:
407:
396:
390:
389:
386:RNA expression
378:
377:
374:
373:
365:
361:
360:
352:
349:
344:
338:
337:
328:
321:
315:
311:
310:
305:
299:
298:
290:
289:
277:
276:
273:
272:
264:
260:
259:
251:
248:
243:
237:
236:
227:
220:
214:
210:
209:
202:
196:
195:
187:
186:
174:
173:
130:
126:
125:
117:
109:
108:
104:
103:
100:
99:
96:
95:
69:
68:
60:
59:
48:
42:
41:
33:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
2624:
2613:
2610:
2608:
2605:
2603:
2600:
2599:
2597:
2586:
2581:
2579:
2574:
2572:
2567:
2566:
2560:
2558:
2554:
2550:
2546:
2541:
2538:
2534:
2529:
2518:
2515:
2513:
2510:
2508:
2505:
2503:
2500:
2496:
2493:
2491:
2488:
2487:
2486:
2483:
2481:
2478:
2476:
2473:
2471:
2468:
2466:
2463:
2461:
2458:
2454:
2451:
2449:
2446:
2444:
2441:
2440:
2439:
2436:
2435:
2433:
2429:
2421:
2418:
2416:
2413:
2411:
2408:
2406:
2403:
2402:
2401:
2398:
2394:
2391:
2389:
2386:
2384:
2381:
2380:
2379:
2376:
2372:
2369:
2367:
2364:
2362:
2359:
2358:
2357:
2354:
2353:
2351:
2349:
2348:Dehydrogenase
2345:
2337:
2334:
2332:
2329:
2328:
2327:
2324:
2322:
2319:
2318:
2316:
2314:
2310:
2306:
2302:
2295:
2290:
2288:
2283:
2281:
2276:
2275:
2272:
2260:
2257:
2256:
2254:
2252:
2248:
2244:
2236:
2233:
2231:
2228:
2226:
2223:
2221:
2218:
2217:
2216:
2213:
2211:
2208:
2206:
2203:
2202:
2200:
2198:
2194:
2190:
2184:
2181:
2180:
2178:
2176:
2172:
2168:
2162:
2159:
2158:
2156:
2154:
2150:
2146:
2140:
2137:
2133:
2130:
2128:
2125:
2124:
2123:
2120:
2119:
2117:
2115:
2111:
2107:
2103:
2098:
2094:
2091:Aldehyde/oxo
2086:
2081:
2079:
2074:
2072:
2067:
2066:
2063:
2049:
2043:
2038:
2034:
2028:
2023:
2019:
2013:
2008:
2004:
1998:
1993:
1989:
1983:
1978:
1974:
1968:
1963:
1959:
1953:
1948:
1944:
1938:
1933:
1929:
1920:
1915:
1913:
1908:
1906:
1901:
1900:
1897:
1888:
1884:
1879:
1874:
1871:(2): 414–24.
1870:
1866:
1862:
1857:
1853:
1849:
1845:
1841:
1837:
1833:
1827:
1823:
1819:
1814:
1809:
1805:
1801:
1797:
1793:
1789:
1784:
1780:
1776:
1771:
1766:
1762:
1758:
1757:J. Biol. Chem
1754:
1749:
1745:
1741:
1736:
1731:
1727:
1723:
1719:
1714:
1710:
1706:
1702:
1698:
1694:
1690:
1685:
1681:
1677:
1673:
1669:
1665:
1661:
1655:
1651:
1647:
1643:
1639:
1635:
1631:
1626:
1622:
1618:
1613:
1608:
1604:
1600:
1599:J. Biol. Chem
1596:
1591:
1587:
1583:
1578:
1573:
1569:
1565:
1561:
1557:
1553:
1548:
1544:
1540:
1535:
1530:
1526:
1522:
1519:(2): 229–32.
1518:
1514:
1510:
1505:
1501:
1497:
1492:
1487:
1484:(2): 342–50.
1483:
1479:
1475:
1470:
1466:
1462:
1458:
1454:
1448:
1444:
1440:
1436:
1432:
1428:
1424:
1418:
1414:
1410:
1406:
1402:
1398:
1394:
1388:
1384:
1380:
1375:
1370:
1366:
1362:
1361:J. Biol. Chem
1358:
1353:
1349:
1345:
1341:
1337:
1332:
1327:
1323:
1319:
1315:
1310:
1309:
1304:
1295:
1289:
1287:
1283:
1278:
1274:
1269:
1264:
1260:
1256:
1252:
1245:
1242:
1237:
1233:
1229:
1225:
1222:(3): 319–21.
1221:
1217:
1210:
1207:
1202:
1198:
1192:
1189:
1184:
1180:
1174:
1171:
1167:
1163:
1158:
1156:
1154:
1150:
1146:
1142:
1137:
1135:
1133:
1129:
1122:
1120:
1118:
1114:
1110:
1106:
1105:dehydrogenase
1102:
1101:decarboxylase
1098:
1094:
1090:
1086:
1082:
1079:(BCKD) is an
1078:
1073:
1071:
1068:
1064:
1060:
1048:
1043:
1039:
1035:
1032:
1028:
1021:
1019:
1016:
1012:
1008:
1004:
1001:
997:
993:
986:
984:
980:
974:
970:
967:
961:
959:
953:
949:
946:
942:
935:
933:
929:
923:
919:
916:
910:
908:
902:
898:
895:
893:RefSeq (mRNA)
891:
884:
883:
878:
874:
871:
865:
864:
859:
855:
852:
850:
846:
839:
838:
833:
829:
826:
820:
819:
814:
810:
807:
805:
801:
794:
793:
788:
784:
781:
775:
774:
769:
765:
762:
760:
756:
753:
750:
748:
745:
741:
738:
734:
730:
723:
719:
714:
708:
705:
703:
700:
699:
697:
694:
693:
687:
684:
682:
681:mitochondrion
679:
677:
674:
672:
669:
667:
664:
663:
661:
658:
657:
651:
648:
646:
643:
641:
638:
636:
633:
631:
628:
626:
623:
622:
620:
617:
616:
613:
612:Gene ontology
609:
605:
593:
588:
584:
577:
570:
565:
562:
560:
556:
548:
543:
532:
528:
524:
520:
516:
512:
508:
504:
501:parotid gland
500:
496:
495:
492:
488:
483:
480:
470:
466:
462:
458:
454:
450:
446:
442:
439:renal medulla
438:
434:
433:
430:
426:
421:
418:
417:
414:
412:
408:
406:
405:
401:
400:
397:
395:
391:
387:
383:
379:
371:
366:
362:
358:
353:
343:
339:
332:
325:
319:
312:
304:
300:
296:
291:
287:
282:
278:
270:
265:
261:
257:
252:
242:
238:
231:
224:
218:
211:
207:
201:
197:
193:
188:
184:
179:
175:
171:
167:
163:
159:
155:
151:
147:
143:
139:
135:
127:
122:
115:
110:
105:
94:
92:
88:
84:
80:
76:
70:
65:
62:
61:
57:
54:
47:
43:
38:
34:
30:
25:
20:
2557:expanding it
2549:chromosome 1
2542:
2527:
2414:
2229:
2047:
2032:
2017:
2002:
1987:
1972:
1957:
1942:
1868:
1864:
1838:(1): 125–8.
1835:
1831:
1798:(1): 59–67.
1795:
1791:
1760:
1756:
1728:(5): 764–9.
1725:
1721:
1695:(2): 961–7.
1692:
1688:
1663:
1660:Biochemistry
1659:
1633:
1629:
1602:
1598:
1559:
1555:
1516:
1512:
1481:
1477:
1459:(1): 49–63.
1456:
1452:
1429:(3): 740–7.
1426:
1422:
1399:(2): 804–9.
1396:
1392:
1364:
1360:
1321:
1317:
1258:
1254:
1244:
1219:
1215:
1209:
1200:
1191:
1182:
1173:
1097:transacylase
1074:
1066:
1058:
1057:
983:NP_001344273
976:
955:
932:NM_001357344
925:
904:
880:
861:
835:
816:
790:
771:
751:
746:
529:right kidney
525:human kidney
409:
402:
368:116,343,630
355:116,306,719
267:100,249,834
254:100,186,919
129:External IDs
72:
2326:Photosystem
1925:PDB gallery
1255:J Biol Chem
107:Identifiers
2596:Categories
2153:cytochrome
1792:Biochem. J
1168:, May 2017
1147:, May 2017
1123:References
1085:isoleucine
702:metabolism
413:(ortholog)
150:HomoloGene
2547:on human
2197:disulfide
1513:FEBS Lett
1109:Mutations
979:NP_034152
958:NP_001909
928:NM_010022
907:NM_001918
737:Orthologs
686:cytoplasm
158:GeneCards
1423:Genomics
1348:25326935
1164:–
1143:–
1031:Wikidata
716:Sources:
2301:Enzymes
1887:8430702
1878:1682180
1852:7918575
1822:6895847
1813:1163502
1779:3245861
1744:3189339
1735:1715554
1709:2932110
1680:2837277
1650:2742576
1621:2708389
1586:2010537
1543:2001734
1521:Bibcode
1500:1990841
1491:1683011
1465:1943690
1443:1889817
1413:1847055
1383:1377677
1340:7672509
1318:FASEB J
1277:1429740
1236:1420314
1166:Ensembl
1145:Ensembl
1089:leucine
849:UniProt
804:Ensembl
743:Species
722:QuickGO
459:pylorus
388:pattern
114:Aliases
2410:BCKDHB
2405:BCKDHA
2225:BCKDHB
2220:BCKDHA
2175:oxygen
1885:
1875:
1850:
1820:
1810:
1777:
1742:
1732:
1707:
1678:
1648:
1619:
1584:
1577:295137
1574:
1541:
1498:
1488:
1463:
1441:
1411:
1381:
1346:
1338:
1275:
1234:
1103:(E1),
1093:valine
1091:, and
1063:enzyme
1061:is an
1017:search
1015:PubMed
882:P53395
863:P11182
759:Entrez
559:BioGPS
455:nipple
447:cardia
246:1p21.2
146:105386
138:248610
2551:is a
2495:HADHB
2490:HADHA
2431:Other
2247:1.2.7
2193:1.2.4
2171:1.2.3
2149:1.2.2
2106:1.2.1
1344:S2CID
792:13171
752:Mouse
747:Human
718:Amigo
411:Mouse
404:Human
351:Start
286:Mouse
250:Start
183:Human
2553:stub
2545:gene
2388:DLST
2383:OGDH
2114:NADP
2099:1.2)
2048:2ii5
2033:2ii4
2018:2ii3
2003:2ihw
1988:2coo
1973:1zwv
1958:1k8o
1943:1k8m
1883:PMID
1848:PMID
1836:1201
1818:PMID
1775:PMID
1740:PMID
1705:PMID
1676:PMID
1646:PMID
1617:PMID
1582:PMID
1539:PMID
1496:PMID
1461:PMID
1439:PMID
1409:PMID
1379:PMID
1336:PMID
1273:PMID
1232:PMID
1220:1132
1075:The
1070:gene
773:1629
394:Bgee
342:Band
303:Chr.
241:Band
200:Chr.
154:1444
134:OMIM
91:3RNM
87:2COO
83:1ZWV
79:1K8O
75:1K8M
56:RCSB
53:PDBe
2438:CAD
2420:DLD
2415:DBT
2393:DLD
2235:DLD
2230:DBT
2112:or
2110:NAD
1873:PMC
1840:doi
1808:PMC
1800:doi
1796:200
1765:doi
1761:263
1730:PMC
1697:doi
1693:131
1668:doi
1638:doi
1634:161
1607:doi
1603:264
1572:PMC
1564:doi
1529:doi
1517:279
1486:PMC
1431:doi
1401:doi
1397:174
1369:doi
1365:267
1326:doi
1263:doi
1259:267
1224:doi
1067:DBT
364:End
263:End
166:OMA
162:DBT
142:MGI
121:DBT
46:PDB
22:DBT
2598::
2371:E3
2366:E2
2361:E1
2336:II
2303::
2249::
2195::
2173::
2151::
2108::
2097:EC
1881:.
1869:52
1867:.
1863:.
1846:.
1834:.
1816:.
1806:.
1794:.
1790:.
1773:.
1759:.
1755:.
1738:.
1726:43
1724:.
1720:.
1703:.
1691:.
1674:.
1664:27
1662:.
1644:.
1632:.
1615:.
1601:.
1597:.
1580:.
1570:.
1560:87
1558:.
1554:.
1537:.
1527:.
1515:.
1511:.
1494:.
1482:48
1480:.
1476:.
1455:.
1437:.
1427:10
1425:.
1407:.
1395:.
1377:.
1363:.
1359:.
1342:.
1334:.
1320:.
1316:.
1285:^
1271:.
1257:.
1253:.
1230:.
1218:.
1199:.
1181:.
1152:^
1131:^
1087:,
1072:.
720:/
370:bp
357:bp
269:bp
256:bp
164:;
160::
156:;
152::
148:;
144::
140:;
136::
89:,
85:,
81:,
77:,
2584:e
2577:t
2570:v
2559:.
2331:I
2293:e
2286:t
2279:v
2095:(
2084:e
2077:t
2070:v
1918:e
1911:t
1904:v
1889:.
1854:.
1842::
1824:.
1802::
1781:.
1767::
1746:.
1711:.
1699::
1682:.
1670::
1652:.
1640::
1623:.
1609::
1588:.
1566::
1545:.
1531::
1523::
1502:.
1467:.
1457:8
1445:.
1433::
1415:.
1403::
1385:.
1371::
1350:.
1328::
1322:9
1296:.
1279:.
1265::
1238:.
1226::
1203:.
1185:.
288:)
185:)
168::
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
