385:
362:
259:
284:
1233:
2423:
2438:
2408:
2453:
2363:
2348:
2333:
636:
2468:
2378:
643:
391:
290:
2393:
40:
2483:
3326:
1250:
66:
1240:
UroD is regarded as an unusual decarboxylase, since it performs decarboxylations without the intervention of any cofactors, unlike the vast majority of decarboxylases. Its mechanism has recently been proposed to proceed through substrate protonation by an
1228:
from the D, A, B, and C rings, whereas at higher substrate/enzyme levels a random route seems to be operative. The enzyme functions as a dimer in solution, and both the enzymes from human and tobacco have been crystallized and solved at good resolutions.
1245:
residue. A 2008 report demonstrated that the uncatalyzed rate for UroD's reaction is 10 s, so at pH 10 the rate acceleration of UroD relative to the uncatalyzed rate, i.e. catalytic proficiency, is the largest for any enzyme known, 6 x 10 M.
1871:"A mutation (G281E) of the human uroporphyrinogen decarboxylase gene causes both hepatoerythropoietic porphyria and overt familial porphyria cutanea tarda: biochemical and genetic studies on Spanish patients"
2221:"Screening for mutations in the uroporphyrinogen decarboxylase gene using denaturing gradient gel electrophoresis. Identification and characterization of six novel mutations associated with familial PCT"
2539:
1191:
2422:
2735:
2437:
2407:
2774:
2452:
398:
297:
2032:
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library".
2362:
2347:
2332:
1553:"Characterization of a new mutation (R292G) and a deletion at the human uroporphyrinogen decarboxylase locus in two patients with hepatoerythropoietic porphyria"
1933:
Meguro K, Fujita H, Ishida N, et al. (1994). "Molecular defects of uroporphyrinogen decarboxylase in a patient with mild hepatoerythropoietic porphyria".
2467:
2377:
2106:"Familial porphyria cutanea tarda: characterization of seven novel uroporphyrinogen decarboxylase mutations and frequency of common hemochromatosis alleles"
1423:
Silva PJ, Ramos MJ (2005). "Density-functional study of mechanisms for the cofactor-free decarboxylation performed by uroporphyrinogen III decarboxylase".
2532:
1832:
de
Verneuil H, Grandchamp B, Beaumont C, et al. (1986). "Uroporphyrinogen decarboxylase structural mutant (Gly281----Glu) in a case of porphyria".
841:
822:
220:
2869:
2893:
2767:
1752:
Dubart A, Mattei MG, Raich N, et al. (1986). "Assignment of human uroporphyrinogen decarboxylase (URO-D) to the p34 band of chromosome 1".
2525:
2312:
1331:
1313:
3346:
2976:
2392:
1904:
Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides".
1602:
Romana M, Grandchamp B, Dubart A, et al. (1991). "Identification of a new mutation responsible for hepatoerythropoietic porphyria".
2874:
2824:
1964:"Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria"
1641:"Uroporphyrinogen decarboxylase: a splice site mutation causes the deletion of exon 6 in multiple families with porphyria cutanea tarda"
2503:
2186:"Three new mutations in the uroporphyrinogen decarboxylase gene in familial porphyria cutanea tarda. Mutation in brief no. 237. Online"
1522:
Elder GH, Lee GB, Tovey JA (1978). "Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda".
384:
1048:
2760:
2202:
2185:
1041:
3045:
2859:
2237:
2220:
361:
2889:
2814:
1352:
2864:
1300:
1279:
2921:
2654:
1213:
3201:
1296:
283:
258:
63:
2629:
1684:"A point mutation in the coding region of uroporphyrinogen decarboxylase associated with familial porphyria cutanea tarda"
3316:
1368:"Refinement of evolutionary medicine predictions based on clinical evidence for the manifestations of Mendelian diseases"
1275:
2943:
2587:
200:
2482:
1224:
At low substrate concentrations, the reaction is believed to follow an ordered route, with the sequential removal of CO
397:
296:
2854:
2809:
2659:
3186:
3302:
3289:
3276:
3263:
3250:
3237:
3224:
2986:
2958:
2908:
2844:
2797:
2624:
2305:
390:
289:
3196:
3150:
3093:
3010:
2788:
2619:
1999:"Five new mutations in the uroporphyrinogen decarboxylase gene identified in families with cutaneous porphyria"
1116:
886:
208:
3098:
2849:
2834:
2829:
2729:
1209:
867:
2517:
2879:
2819:
1193:
1164:
1176:
3119:
3038:
3191:
2147:
Wang H, Long Q, Marty SD, et al. (1998). "A zebrafish model for hepatoerythropoietic porphyria".
2839:
2707:
2298:
1841:
1471:
1379:
1171:
1160:
272:
187:
3155:
1719:"Molecular cloning and nucleotide sequence of a complete human uroporphyrinogen decarboxylase cDNA"
1232:
3088:
2948:
2552:
2250:
2172:
2015:
1998:
1777:
1627:
1590:
232:
1024:
1003:
977:
956:
3351:
2270:
2242:
2207:
2164:
2135:
2092:
2049:
2020:
1985:
1950:
1921:
1892:
1857:
1820:
1769:
1740:
1705:
1670:
1619:
1582:
1539:
1499:
1440:
1405:
1139:
180:
56:
3134:
3129:
3103:
3031:
2971:
2232:
2197:
2156:
2125:
2117:
2082:
2074:
2041:
2010:
1975:
1942:
1913:
1882:
1849:
1810:
1802:
1761:
1730:
1695:
1660:
1652:
1611:
1572:
1564:
1531:
1489:
1479:
1432:
1395:
1387:
635:
477:
408:
352:
307:
3181:
3165:
3078:
2431:: Uroporphyrinogen Decarboxylase single mutant D86G in complex with coproporphyrinogen-III
1460:"Uroporphyrinogen decarboxylation as a benchmark for the catalytic proficiency of enzymes"
642:
452:
228:
2446:: Uroporphyrinogen Decarboxylase single mutant D86E in complex with coproporphyrinogen-I
2416:: Uroporphyrinogen Decarboxylase single mutant D86G in complex with coproporphyrinogen-I
1845:
1475:
1383:
1119:
3330:
3219:
3160:
2801:
2702:
2664:
2130:
2105:
2087:
2062:
1980:
1963:
1615:
1494:
1459:
1400:
1367:
2045:
1815:
1790:
1735:
1718:
1665:
1640:
756:
751:
746:
741:
725:
720:
715:
699:
694:
689:
684:
39:
3340:
3124:
3083:
2995:
2511:
2279:
1917:
671:
2461:: Uroporphyrinogen Decarboxylase Y164F mutant in complex with coproporphyrinogen-III
2176:
1781:
1631:
1594:
3073:
470:
249:
2254:
212:
1946:
1887:
1870:
236:
3297:
3232:
3068:
2752:
1700:
1683:
1535:
1336:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1318:
National Center for
Biotechnology Information, U.S. National Library of Medicine
3325:
1391:
553:
3000:
2936:
2931:
2926:
2078:
369:
266:
216:
1806:
3271:
3245:
3005:
2568:
2560:
2507:
2502:
1853:
1484:
1249:
786:
613:
491:
436:
423:
335:
322:
224:
2246:
2211:
1503:
1444:
1409:
1216:. At least 65 disease-causing mutations in this gene have been discovered.
2371:: Gly156Asp mutant of Human UroD, human uroporphyrinogen III decarboxylase
2356:: Phe232Leu mutant of human UROD, human uroporphyrinogen III decarboxylase
2341:: Ile260Thr mutant of Human UroD, human uroporphyrinogen III decarboxylase
2168:
2139:
2096:
2053:
2024:
1989:
1954:
1925:
1896:
1861:
1824:
1773:
1744:
1709:
1674:
1623:
1586:
1088:
1083:
2912:
1543:
1242:
1152:
1072:
931:
912:
2203:
10.1002/(SICI)1098-1004(1999)13:5<412::AID-HUMU13>3.0.CO;2-N
2962:
2884:
2476:: Uroporphyrinogen Decarboxylase in complex with coproporphyrinogen-III
2284:
2274:
2238:
10.1002/(SICI)1098-1004(1999)14:3<222::AID-HUMU5>3.0.CO;2-V
1765:
1568:
1156:
898:
853:
1656:
1577:
1436:
1143:
157:
153:
149:
145:
141:
137:
133:
129:
125:
121:
117:
113:
109:
105:
101:
97:
93:
89:
85:
3284:
3054:
2548:
2386:: Uroporphyrinogen Decarboxylase in complex with coproporphyrinogen-I
1112:
1056:
808:
1208:
Mutations and deficiency in this enzyme are known to cause familial
17:
2121:
3258:
2784:
2597:
2592:
2501:
1248:
1231:
2160:
1253:
Proposed reaction mechanism of uroporphyrinogen III decarboxylase
771:
767:
2683:
2678:
2556:
1791:"Structure of the gene for human uroporphyrinogen decarboxylase"
1552:
1148:
1126:
204:
3027:
2756:
2521:
2294:
651:
2290:
2269:
Overview of all the structural information available in the
1551:
de
Verneuil H, Bourgeois F, de Rooij F, et al. (1992).
1137:
Uroporphyrinogen III decarboxylase is a homodimeric enzyme (
2063:"Crystal structure of human uroporphyrinogen decarboxylase"
1869:
Roberts AG, Elder GH, De
Salamanca RE, et al. (1995).
3023:
2219:
Christiansen L, Ged C, Hombrados I, et al. (1999).
1639:
Garey JR, Harrison LM, Franklin KF, et al. (1990).
1180:
460:
1962:
Moran-Jimenez MJ, Ged C, Romana M, et al. (1996).
1682:
Garey JR, Hansen JL, Harrison LM, et al. (1989).
1151:
biosynthesis, which corresponds to the elimination of
3314:
2506:
Heme synthesis—note that some reactions occur in the
2104:
Mendez M, Sorkin L, Rossetti MV, et al. (1998).
1179:
625:
2061:
Whitby FG, Phillips JD, Kushner JP, Hill CP (1998).
1789:
Romana M, Dubart A, Beaupain D, et al. (1987).
3210:
3174:
3143:
3112:
3061:
2985:
2957:
2907:
2796:
2717:
2691:
2677:
2643:
2608:
2576:
2567:
2401:: Uroporphyrinogen Decarboxylase with mutation D86N
2184:McManus JF, Begley CG, Sassa S, Ratnaike S (1999).
1997:McManus JF, Begley CG, Sassa S, Ratnaike S (1996).
1017:
996:
970:
949:
1353:"Entrez Gene: UROD uroporphyrinogen decarboxylase"
1292:
1290:
1288:
1271:
1269:
1267:
1185:
747:porphyrin-containing compound biosynthetic process
1717:Roméo PH, Raich N, Dubart A, et al. (1986).
407:
306:
1297:GRCm38: Ensembl release 89: ENSMUSG00000028684
3039:
2768:
2533:
2306:
8:
2016:10.1182/blood.V88.9.3589.bloodjournal8893589
1276:GRCh38: Ensembl release 89: ENSG00000126088
3046:
3032:
3024:
2775:
2761:
2753:
2688:
2573:
2540:
2526:
2518:
2313:
2299:
2291:
1147:) that catalyzes the fifth step in
782:
742:protoporphyrinogen IX biosynthetic process
667:
448:
347:
244:
191:, PCT, UPD, uroporphyrinogen decarboxylase
74:
2236:
2201:
2129:
2086:
2014:
1979:
1886:
1814:
1734:
1699:
1664:
1576:
1493:
1483:
1399:
1366:Ĺ imÄŤĂková D, Heneberg P (December 2019).
1178:
564:fetal liver hematopoietic progenitor cell
2870:Phosphoribosylaminoimidazole carboxylase
2283:(Uroporphyrinogen decarboxylase) at the
1347:
1345:
3321:
2328:
1458:Lewis CA, Wolfenden R (November 2008).
1263:
695:uroporphyrinogen decarboxylase activity
27:Mammalian protein found in Homo sapiens
29:
412:
373:
368:
311:
270:
265:
7:
2977:3-hydroxy-3-methylglutaryl-CoA lyase
2894:Orotidine 5'-phosphate decarboxylase
2875:Pyrophosphomevalonate decarboxylase
2825:Aromatic L-amino acid decarboxylase
1186:{\displaystyle \rightleftharpoons }
1122:) that in humans is encoded by the
2899:Uroporphyrinogen III decarboxylase
2635:Uroporphyrinogen III decarboxylase
1616:10.1111/j.1365-2362.1991.tb01814.x
1101:Uroporphyrinogen III decarboxylase
1014:
993:
967:
946:
922:
903:
877:
858:
832:
813:
630:
548:
486:
465:
25:
2860:Phosphoenolpyruvate carboxykinase
3324:
2890:Uridine monophosphate synthetase
2815:Adenosylmethionine decarboxylase
2491:: UROPORPHYRINOGEN DECARBOXYLASE
2481:
2466:
2451:
2436:
2421:
2406:
2391:
2376:
2361:
2346:
2331:
641:
634:
396:
389:
383:
360:
295:
288:
282:
257:
38:
2865:Phosphoenolpyruvate carboxylase
2922:Fructose-bisphosphate aldolase
2655:Coproporphyrinogen III oxidase
1236:The reaction catalyzed by UroD
1214:hepatoerythropoietic porphyria
1105:uroporphyrinogen decarboxylase
652:More reference expression data
614:More reference expression data
1:
2630:Uroporphyrinogen III synthase
2046:10.1016/S0378-1119(97)00411-3
1736:10.1016/S0021-9258(18)67589-1
381:
280:
2944:2-hydroxyphytanoyl-CoA lyase
2588:Aminolevulinic acid synthase
1947:10.1111/1523-1747.ep12374134
1918:10.1016/0378-1119(94)90802-8
1888:10.1111/1523-1747.ep12605953
1464:Proc. Natl. Acad. Sci. U.S.A
592:Epithelium of choroid plexus
3347:Genes on human chromosome 1
1701:10.1182/blood.V73.4.892.892
1536:10.1056/NEJM197808102990603
3368:
2855:Oxaloacetate decarboxylase
2810:Acetoacetate decarboxylase
2660:Protoporphyrinogen oxidase
1392:10.1038/s41598-019-54976-4
3202:Michaelis–Menten kinetics
2845:Malonyl-CoA decarboxylase
2625:Porphobilinogen deaminase
2326:
1332:"Mouse PubMed Reference:"
1314:"Human PubMed Reference:"
1087:
1082:
1078:
1071:
1055:
1049:Chr 4: 116.85 – 116.85 Mb
1036:
1021:
1000:
989:
974:
953:
942:
929:
925:
910:
906:
897:
884:
880:
865:
861:
852:
839:
835:
820:
816:
807:
792:
785:
781:
765:
752:heme biosynthetic process
670:
666:
649:
633:
624:
611:
596:medullary collecting duct
560:
551:
498:
489:
459:
451:
447:
430:
417:
380:
359:
350:
346:
329:
316:
279:
256:
247:
243:
198:
195:
185:
178:
173:
82:
77:
60:
55:
50:
46:
37:
32:
3094:Diffusion-limited enzyme
3011:Spore photoproduct lyase
2620:Porphobilinogen synthase
2850:Ornithine decarboxylase
2835:Histidine decarboxylase
2830:Glutamate decarboxylase
2730:glucuronosyltransferase
2079:10.1093/emboj/17.9.2463
1854:10.1126/science.3775362
1485:10.1073/pnas.0809838105
1210:porphyria cutanea tarda
1042:Chr 1: 45.01 – 45.02 Mb
580:external carotid artery
568:internal carotid artery
2880:Pyruvate decarboxylase
2820:Arginine decarboxylase
2569:Porphyrin biosynthesis
2515:
1807:10.1093/nar/15.18.7343
1254:
1237:
1194:coproporphyrinogen III
1187:
1165:coproporphyrinogen III
757:heme metabolic process
700:carboxy-lyase activity
588:atrioventricular valve
3187:Eadie–Hofstee diagram
3120:Allosteric regulation
2505:
1252:
1235:
1204:Clinical significance
1188:
1155:groups from the four
530:middle temporal gyrus
3197:Lineweaver–Burk plot
2840:Lysine decarboxylase
2708:Biliverdin reductase
2579:early mitochondrial:
1604:Eur. J. Clin. Invest
1177:
1172:uroporphyrinogen III
1161:uroporphyrinogen III
584:facial motor nucleus
514:right adrenal cortex
414:4 D1|4 53.41 cM
375:Chromosome 4 (mouse)
273:Chromosome 1 (human)
78:List of PDB id codes
51:Available structures
2646:late mitochondrial:
1935:J. Invest. Dermatol
1875:J. Invest. Dermatol
1846:1986Sci...234..732D
1476:2008PNAS..10517328L
1384:2019NatSR...918577S
572:endocardial cushion
522:left adrenal cortex
510:right adrenal gland
3156:Enzyme superfamily
3089:Enzyme promiscuity
2949:Threonine aldolase
2516:
1766:10.1007/BF00401245
1569:10.1007/bf00219182
1372:Scientific Reports
1255:
1238:
1183:
887:ENSMUSG00000028684
735:Biological process
709:Cellular component
678:Molecular function
3312:
3311:
3021:
3020:
2750:
2749:
2746:
2745:
2673:
2672:
2499:
2498:
2110:Am. J. Hum. Genet
1968:Am. J. Hum. Genet
1795:Nucleic Acids Res
1657:10.1172/JCI114856
1437:10.1021/jp051792s
1431:(38): 18195–200.
1098:
1097:
1094:
1093:
1067:
1066:
1032:
1031:
1011:
1010:
985:
984:
964:
963:
938:
937:
919:
918:
893:
892:
874:
873:
848:
847:
829:
828:
777:
776:
662:
661:
658:
657:
620:
619:
607:
606:
545:
544:
443:
442:
342:
341:
169:
168:
165:
164:
61:Ortholog search:
16:(Redirected from
3359:
3329:
3328:
3320:
3192:Hanes–Woolf plot
3135:Enzyme activator
3130:Enzyme inhibitor
3104:Enzyme catalysis
3048:
3041:
3034:
3025:
2972:Isocitrate lyase
2777:
2770:
2763:
2754:
2722:
2696:
2689:
2679:Heme degradation
2648:
2613:
2581:
2574:
2551:involved in the
2542:
2535:
2528:
2519:
2510:and some in the
2485:
2470:
2455:
2440:
2425:
2410:
2395:
2380:
2365:
2350:
2335:
2315:
2308:
2301:
2292:
2258:
2240:
2215:
2205:
2180:
2143:
2133:
2100:
2090:
2057:
2028:
2018:
1993:
1983:
1958:
1929:
1900:
1890:
1865:
1828:
1818:
1785:
1748:
1738:
1713:
1703:
1678:
1668:
1635:
1598:
1580:
1547:
1508:
1507:
1497:
1487:
1470:(45): 17328–33.
1455:
1449:
1448:
1420:
1414:
1413:
1403:
1363:
1357:
1356:
1349:
1340:
1339:
1328:
1322:
1321:
1310:
1304:
1294:
1283:
1273:
1192:
1190:
1189:
1184:
1146:
1080:
1079:
1051:
1044:
1027:
1015:
1006:
994:
990:RefSeq (protein)
980:
968:
959:
947:
923:
904:
878:
859:
833:
814:
783:
668:
654:
645:
638:
631:
616:
556:
554:Top expressed in
549:
534:Brodmann area 23
526:endothelial cell
494:
492:Top expressed in
487:
466:
449:
439:
426:
415:
400:
393:
387:
376:
364:
348:
338:
325:
314:
299:
292:
286:
275:
261:
245:
239:
237:UROD - orthologs
190:
183:
160:
75:
69:
48:
47:
42:
30:
21:
3367:
3366:
3362:
3361:
3360:
3358:
3357:
3356:
3337:
3336:
3335:
3323:
3315:
3313:
3308:
3220:Oxidoreductases
3206:
3182:Enzyme kinetics
3170:
3166:List of enzymes
3139:
3108:
3079:Catalytic triad
3057:
3052:
3022:
3017:
2981:
2953:
2903:
2792:
2781:
2751:
2742:
2718:
2713:
2710:
2692:
2681:
2669:
2644:
2639:
2609:
2604:
2577:
2563:
2546:
2500:
2495:
2492:
2486:
2477:
2471:
2462:
2456:
2447:
2441:
2432:
2426:
2417:
2411:
2402:
2396:
2387:
2381:
2372:
2366:
2357:
2351:
2342:
2336:
2322:
2319:
2266:
2261:
2218:
2183:
2146:
2103:
2060:
2040:(1–2): 149–56.
2031:
2009:(9): 3589–600.
1996:
1961:
1932:
1903:
1868:
1840:(4777): 732–4.
1831:
1801:(18): 7343–56.
1788:
1751:
1729:(21): 9825–31.
1716:
1681:
1645:J. Clin. Invest
1638:
1601:
1550:
1524:N. Engl. J. Med
1521:
1517:
1515:Further reading
1512:
1511:
1457:
1456:
1452:
1422:
1421:
1417:
1365:
1364:
1360:
1351:
1350:
1343:
1330:
1329:
1325:
1312:
1311:
1307:
1295:
1286:
1274:
1265:
1260:
1227:
1222:
1206:
1199:
1175:
1174:
1159:side chains of
1138:
1135:
1089:View/Edit Mouse
1084:View/Edit Human
1047:
1040:
1037:Location (UCSC)
1023:
1002:
976:
955:
868:ENSG00000126088
761:
730:
704:
685:protein binding
650:
640:
639:
612:
603:
600:renal corpuscle
598:
594:
590:
586:
582:
578:
574:
570:
566:
552:
541:
536:
532:
528:
524:
520:
518:parietal pleura
516:
512:
508:
504:
502:trabecular bone
490:
434:
421:
413:
403:
402:
401:
394:
374:
351:Gene location (
333:
320:
312:
302:
301:
300:
293:
271:
248:Gene location (
199:
186:
179:
84:
62:
28:
23:
22:
15:
12:
11:
5:
3365:
3363:
3355:
3354:
3349:
3339:
3338:
3334:
3333:
3310:
3309:
3307:
3306:
3293:
3280:
3267:
3254:
3241:
3228:
3214:
3212:
3208:
3207:
3205:
3204:
3199:
3194:
3189:
3184:
3178:
3176:
3172:
3171:
3169:
3168:
3163:
3158:
3153:
3147:
3145:
3144:Classification
3141:
3140:
3138:
3137:
3132:
3127:
3122:
3116:
3114:
3110:
3109:
3107:
3106:
3101:
3096:
3091:
3086:
3081:
3076:
3071:
3065:
3063:
3059:
3058:
3053:
3051:
3050:
3043:
3036:
3028:
3019:
3018:
3016:
3015:
3014:
3013:
3008:
2998:
2992:
2990:
2983:
2982:
2980:
2979:
2974:
2968:
2966:
2955:
2954:
2952:
2951:
2946:
2941:
2940:
2939:
2934:
2929:
2918:
2916:
2905:
2904:
2902:
2901:
2896:
2887:
2882:
2877:
2872:
2867:
2862:
2857:
2852:
2847:
2842:
2837:
2832:
2827:
2822:
2817:
2812:
2806:
2804:
2802:Carboxy-lyases
2794:
2793:
2783:Carbon–carbon
2782:
2780:
2779:
2772:
2765:
2757:
2748:
2747:
2744:
2743:
2741:
2740:
2739:
2738:
2725:
2723:
2715:
2714:
2712:
2711:
2705:
2703:Heme oxygenase
2699:
2697:
2686:
2675:
2674:
2671:
2670:
2668:
2667:
2665:Ferrochelatase
2662:
2657:
2651:
2649:
2641:
2640:
2638:
2637:
2632:
2627:
2622:
2616:
2614:
2606:
2605:
2603:
2602:
2601:
2600:
2595:
2584:
2582:
2571:
2565:
2564:
2547:
2545:
2544:
2537:
2530:
2522:
2497:
2496:
2494:
2493:
2487:
2480:
2478:
2472:
2465:
2463:
2457:
2450:
2448:
2442:
2435:
2433:
2427:
2420:
2418:
2412:
2405:
2403:
2397:
2390:
2388:
2382:
2375:
2373:
2367:
2360:
2358:
2352:
2345:
2343:
2337:
2330:
2327:
2324:
2323:
2320:
2318:
2317:
2310:
2303:
2295:
2289:
2288:
2265:
2264:External links
2262:
2260:
2259:
2216:
2196:(5): 412–413.
2181:
2144:
2122:10.1086/302119
2116:(5): 1363–75.
2101:
2073:(9): 2463–71.
2058:
2029:
1994:
1959:
1930:
1912:(1–2): 171–4.
1901:
1866:
1829:
1786:
1749:
1714:
1679:
1651:(5): 1416–22.
1636:
1599:
1548:
1518:
1516:
1513:
1510:
1509:
1450:
1415:
1358:
1341:
1323:
1305:
1284:
1262:
1261:
1259:
1256:
1225:
1221:
1218:
1205:
1202:
1201:
1200:
1197:
1182:
1134:
1131:
1096:
1095:
1092:
1091:
1086:
1076:
1075:
1069:
1068:
1065:
1064:
1062:
1060:
1053:
1052:
1045:
1038:
1034:
1033:
1030:
1029:
1019:
1018:
1012:
1009:
1008:
998:
997:
991:
987:
986:
983:
982:
972:
971:
965:
962:
961:
951:
950:
944:
940:
939:
936:
935:
927:
926:
920:
917:
916:
908:
907:
901:
895:
894:
891:
890:
882:
881:
875:
872:
871:
863:
862:
856:
850:
849:
846:
845:
837:
836:
830:
827:
826:
818:
817:
811:
805:
804:
799:
794:
790:
789:
779:
778:
775:
774:
763:
762:
760:
759:
754:
749:
744:
738:
736:
732:
731:
729:
728:
723:
718:
712:
710:
706:
705:
703:
702:
697:
692:
690:lyase activity
687:
681:
679:
675:
674:
664:
663:
660:
659:
656:
655:
647:
646:
628:
622:
621:
618:
617:
609:
608:
605:
604:
602:
601:
597:
593:
589:
585:
581:
577:
573:
569:
565:
561:
558:
557:
546:
543:
542:
540:
539:
535:
531:
527:
523:
519:
515:
511:
507:
503:
499:
496:
495:
483:
482:
474:
463:
457:
456:
453:RNA expression
445:
444:
441:
440:
432:
428:
427:
419:
416:
411:
405:
404:
395:
388:
382:
378:
377:
372:
366:
365:
357:
356:
344:
343:
340:
339:
331:
327:
326:
318:
315:
310:
304:
303:
294:
287:
281:
277:
276:
269:
263:
262:
254:
253:
241:
240:
197:
193:
192:
184:
176:
175:
171:
170:
167:
166:
163:
162:
80:
79:
71:
70:
59:
53:
52:
44:
43:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3364:
3353:
3350:
3348:
3345:
3344:
3342:
3332:
3327:
3322:
3318:
3304:
3300:
3299:
3294:
3291:
3287:
3286:
3281:
3278:
3274:
3273:
3268:
3265:
3261:
3260:
3255:
3252:
3248:
3247:
3242:
3239:
3235:
3234:
3229:
3226:
3222:
3221:
3216:
3215:
3213:
3209:
3203:
3200:
3198:
3195:
3193:
3190:
3188:
3185:
3183:
3180:
3179:
3177:
3173:
3167:
3164:
3162:
3161:Enzyme family
3159:
3157:
3154:
3152:
3149:
3148:
3146:
3142:
3136:
3133:
3131:
3128:
3126:
3125:Cooperativity
3123:
3121:
3118:
3117:
3115:
3111:
3105:
3102:
3100:
3097:
3095:
3092:
3090:
3087:
3085:
3084:Oxyanion hole
3082:
3080:
3077:
3075:
3072:
3070:
3067:
3066:
3064:
3060:
3056:
3049:
3044:
3042:
3037:
3035:
3030:
3029:
3026:
3012:
3009:
3007:
3004:
3003:
3002:
2999:
2997:
2996:Tryptophanase
2994:
2993:
2991:
2988:
2984:
2978:
2975:
2973:
2970:
2969:
2967:
2964:
2960:
2956:
2950:
2947:
2945:
2942:
2938:
2935:
2933:
2930:
2928:
2925:
2924:
2923:
2920:
2919:
2917:
2914:
2910:
2906:
2900:
2897:
2895:
2891:
2888:
2886:
2883:
2881:
2878:
2876:
2873:
2871:
2868:
2866:
2863:
2861:
2858:
2856:
2853:
2851:
2848:
2846:
2843:
2841:
2838:
2836:
2833:
2831:
2828:
2826:
2823:
2821:
2818:
2816:
2813:
2811:
2808:
2807:
2805:
2803:
2799:
2795:
2790:
2786:
2778:
2773:
2771:
2766:
2764:
2759:
2758:
2755:
2737:
2734:
2733:
2732:
2731:
2727:
2726:
2724:
2721:
2716:
2709:
2706:
2704:
2701:
2700:
2698:
2695:
2690:
2687:
2685:
2680:
2676:
2666:
2663:
2661:
2658:
2656:
2653:
2652:
2650:
2647:
2642:
2636:
2633:
2631:
2628:
2626:
2623:
2621:
2618:
2617:
2615:
2612:
2607:
2599:
2596:
2594:
2591:
2590:
2589:
2586:
2585:
2583:
2580:
2575:
2572:
2570:
2566:
2562:
2558:
2554:
2550:
2543:
2538:
2536:
2531:
2529:
2524:
2523:
2520:
2513:
2512:mitochondrion
2509:
2504:
2490:
2484:
2479:
2475:
2469:
2464:
2460:
2454:
2449:
2445:
2439:
2434:
2430:
2424:
2419:
2415:
2409:
2404:
2400:
2394:
2389:
2385:
2379:
2374:
2370:
2364:
2359:
2355:
2349:
2344:
2340:
2334:
2329:
2325:
2316:
2311:
2309:
2304:
2302:
2297:
2296:
2293:
2286:
2282:
2281:
2276:
2272:
2268:
2267:
2263:
2256:
2252:
2248:
2244:
2239:
2234:
2231:(3): 222–32.
2230:
2226:
2222:
2217:
2213:
2209:
2204:
2199:
2195:
2191:
2187:
2182:
2178:
2174:
2170:
2166:
2162:
2158:
2155:(3): 239–43.
2154:
2150:
2145:
2141:
2137:
2132:
2127:
2123:
2119:
2115:
2111:
2107:
2102:
2098:
2094:
2089:
2084:
2080:
2076:
2072:
2068:
2064:
2059:
2055:
2051:
2047:
2043:
2039:
2035:
2030:
2026:
2022:
2017:
2012:
2008:
2004:
2000:
1995:
1991:
1987:
1982:
1977:
1974:(4): 712–21.
1973:
1969:
1965:
1960:
1956:
1952:
1948:
1944:
1940:
1936:
1931:
1927:
1923:
1919:
1915:
1911:
1907:
1902:
1898:
1894:
1889:
1884:
1880:
1876:
1872:
1867:
1863:
1859:
1855:
1851:
1847:
1843:
1839:
1835:
1830:
1826:
1822:
1817:
1812:
1808:
1804:
1800:
1796:
1792:
1787:
1783:
1779:
1775:
1771:
1767:
1763:
1759:
1755:
1750:
1746:
1742:
1737:
1732:
1728:
1724:
1723:J. Biol. Chem
1720:
1715:
1711:
1707:
1702:
1697:
1693:
1689:
1685:
1680:
1676:
1672:
1667:
1662:
1658:
1654:
1650:
1646:
1642:
1637:
1633:
1629:
1625:
1621:
1617:
1613:
1609:
1605:
1600:
1596:
1592:
1588:
1584:
1579:
1574:
1570:
1566:
1563:(5): 548–52.
1562:
1558:
1554:
1549:
1545:
1541:
1537:
1533:
1529:
1525:
1520:
1519:
1514:
1505:
1501:
1496:
1491:
1486:
1481:
1477:
1473:
1469:
1465:
1461:
1454:
1451:
1446:
1442:
1438:
1434:
1430:
1426:
1425:J Phys Chem B
1419:
1416:
1411:
1407:
1402:
1397:
1393:
1389:
1385:
1381:
1377:
1373:
1369:
1362:
1359:
1354:
1348:
1346:
1342:
1337:
1333:
1327:
1324:
1319:
1315:
1309:
1306:
1302:
1298:
1293:
1291:
1289:
1285:
1281:
1277:
1272:
1270:
1268:
1264:
1257:
1251:
1247:
1244:
1234:
1230:
1219:
1217:
1215:
1211:
1203:
1195:
1173:
1170:
1169:
1168:
1166:
1162:
1158:
1154:
1150:
1145:
1141:
1132:
1130:
1128:
1125:
1121:
1118:
1114:
1110:
1106:
1102:
1090:
1085:
1081:
1077:
1074:
1070:
1063:
1061:
1058:
1054:
1050:
1046:
1043:
1039:
1035:
1028:
1026:
1020:
1016:
1013:
1007:
1005:
999:
995:
992:
988:
981:
979:
973:
969:
966:
960:
958:
952:
948:
945:
943:RefSeq (mRNA)
941:
934:
933:
928:
924:
921:
915:
914:
909:
905:
902:
900:
896:
889:
888:
883:
879:
876:
870:
869:
864:
860:
857:
855:
851:
844:
843:
838:
834:
831:
825:
824:
819:
815:
812:
810:
806:
803:
800:
798:
795:
791:
788:
784:
780:
773:
769:
764:
758:
755:
753:
750:
748:
745:
743:
740:
739:
737:
734:
733:
727:
724:
722:
719:
717:
714:
713:
711:
708:
707:
701:
698:
696:
693:
691:
688:
686:
683:
682:
680:
677:
676:
673:
672:Gene ontology
669:
665:
653:
648:
644:
637:
632:
629:
627:
623:
615:
610:
599:
595:
591:
587:
583:
579:
575:
571:
567:
563:
562:
559:
555:
550:
547:
537:
533:
529:
525:
521:
517:
513:
509:
506:parotid gland
505:
501:
500:
497:
493:
488:
485:
484:
481:
479:
475:
473:
472:
468:
467:
464:
462:
458:
454:
450:
446:
438:
433:
429:
425:
420:
410:
406:
399:
392:
386:
379:
371:
367:
363:
358:
354:
349:
345:
337:
332:
328:
324:
319:
309:
305:
298:
291:
285:
278:
274:
268:
264:
260:
255:
251:
246:
242:
238:
234:
230:
226:
222:
218:
214:
210:
206:
202:
194:
189:
182:
177:
172:
161:
159:
155:
151:
147:
143:
139:
135:
131:
127:
123:
119:
115:
111:
107:
103:
99:
95:
91:
87:
81:
76:
73:
72:
68:
65:
58:
54:
49:
45:
41:
36:
31:
19:
3298:Translocases
3295:
3282:
3269:
3256:
3243:
3233:Transferases
3230:
3217:
3074:Binding site
2898:
2728:
2719:
2693:
2645:
2634:
2610:
2578:
2488:
2473:
2458:
2443:
2428:
2413:
2398:
2383:
2368:
2353:
2338:
2278:
2228:
2224:
2193:
2189:
2161:10.1038/3041
2152:
2148:
2113:
2109:
2070:
2066:
2037:
2033:
2006:
2002:
1971:
1967:
1941:(5): 681–5.
1938:
1934:
1909:
1905:
1881:(4): 500–2.
1878:
1874:
1837:
1833:
1798:
1794:
1760:(3): 277–9.
1757:
1753:
1726:
1722:
1694:(4): 892–5.
1691:
1687:
1648:
1644:
1610:(2): 225–9.
1607:
1603:
1560:
1556:
1530:(6): 274–8.
1527:
1523:
1467:
1463:
1453:
1428:
1424:
1418:
1378:(1): 18577.
1375:
1371:
1361:
1335:
1326:
1317:
1308:
1239:
1223:
1207:
1136:
1123:
1108:
1104:
1100:
1099:
1022:
1001:
975:
954:
930:
911:
885:
866:
840:
821:
801:
796:
476:
469:
435:116,851,610
422:116,847,162
196:External IDs
83:
3069:Active site
2321:PDB gallery
716:nucleoplasm
576:human fetus
538:bone marrow
334:45,015,575
321:45,010,950
174:Identifiers
3341:Categories
3272:Isomerases
3246:Hydrolases
3113:Regulation
3001:Photolyase
2937:Aldolase C
2932:Aldolase B
2927:Aldolase A
2611:cytosolic:
2553:metabolism
2225:Hum. Mutat
2190:Hum. Mutat
2149:Nat. Genet
1754:Hum. Genet
1578:1765/58484
1557:Hum. Genet
1303:, May 2017
1282:, May 2017
1258:References
480:(ortholog)
217:HomoloGene
3151:EC number
3006:CPD lyase
2561:porphyrin
2508:cytoplasm
1220:Mechanism
1181:⇌
1163:to yield
1025:NP_033504
1004:NP_000365
978:NM_009478
957:NM_000374
787:Orthologs
721:cytoplasm
225:GeneCards
3352:EC 4.1.1
3175:Kinetics
3099:Cofactor
3062:Activity
2963:Oxo-acid
2913:Aldehyde
2514:(yellow)
2247:10477430
2212:10338097
2177:28379777
1782:34478515
1632:22358220
1595:31811381
1504:18988736
1445:16853337
1410:31819097
1299:–
1278:–
1243:arginine
1153:carboxyl
1133:Function
1120:4.1.1.37
1111:) is an
1073:Wikidata
766:Sources:
3331:Biology
3285:Ligases
3055:Enzymes
2989:: Other
2965:-lyases
2915:-lyases
2885:RuBisCO
2694:spleen:
2549:Enzymes
2285:PDBe-KB
2275:UniProt
2169:9806541
2140:9792863
2131:1377546
2097:9564029
2088:1170588
2054:9373149
2025:8896428
1990:8644733
1981:1914669
1955:8176248
1926:8125298
1897:7706766
1862:3775362
1842:Bibcode
1834:Science
1825:3658695
1774:3460962
1745:3015909
1710:2920211
1675:2243121
1624:1905636
1587:1634232
1495:2582308
1472:Bibcode
1401:6901466
1380:Bibcode
1301:Ensembl
1280:Ensembl
1157:acetate
899:UniProt
854:Ensembl
793:Species
772:QuickGO
726:cytosol
455:pattern
181:Aliases
3317:Portal
3259:Lyases
2987:4.1.99
2785:lyases
2736:UGT1A1
2720:liver:
2280:P06132
2255:245442
2253:
2245:
2210:
2175:
2167:
2138:
2128:
2095:
2085:
2067:EMBO J
2052:
2023:
1988:
1978:
1953:
1924:
1895:
1860:
1823:
1816:306252
1813:
1780:
1772:
1743:
1708:
1673:
1666:296884
1663:
1630:
1622:
1593:
1585:
1544:661926
1542:
1502:
1492:
1443:
1408:
1398:
1196:+ 4 CO
1113:enzyme
1059:search
1057:PubMed
932:P70697
913:P06132
809:Entrez
626:BioGPS
313:1p34.1
205:613521
3211:Types
2959:4.1.3
2909:4.1.2
2798:4.1.1
2598:ALAS2
2593:ALAS1
2251:S2CID
2173:S2CID
2003:Blood
1778:S2CID
1688:Blood
1628:S2CID
1591:S2CID
1107:, or
842:22275
802:Mouse
797:Human
768:Amigo
478:Mouse
471:Human
418:Start
353:Mouse
317:Start
250:Human
213:98916
3303:list
3296:EC7
3290:list
3283:EC6
3277:list
3270:EC5
3264:list
3257:EC4
3251:list
3244:EC3
3238:list
3231:EC2
3225:list
3218:EC1
2791:4.1)
2684:bile
2559:and
2557:heme
2489:1uro
2474:1r3y
2459:1r3w
2444:1r3v
2429:1r3t
2414:1r3s
2399:1r3r
2384:1r3q
2369:1jpk
2354:1jpi
2339:1jph
2273:for
2243:PMID
2208:PMID
2165:PMID
2136:PMID
2093:PMID
2050:PMID
2034:Gene
2021:PMID
1986:PMID
1951:PMID
1922:PMID
1906:Gene
1893:PMID
1858:PMID
1821:PMID
1770:PMID
1741:PMID
1706:PMID
1671:PMID
1620:PMID
1583:PMID
1540:PMID
1500:PMID
1441:PMID
1406:PMID
1212:and
1149:heme
1144:1URO
1127:gene
1124:UROD
1109:UROD
823:7389
461:Bgee
409:Band
370:Chr.
308:Band
267:Chr.
229:UROD
201:OMIM
188:UROD
158:3GW3
154:3GW0
150:3GVW
146:3GVV
142:3GVR
138:3GVQ
134:2Q71
130:2Q6Z
126:1URO
122:1R3Y
118:1R3W
114:1R3V
110:1R3T
106:1R3S
102:1R3R
98:1R3Q
94:1JPK
90:1JPI
86:1JPH
67:RCSB
64:PDBe
33:UROD
18:UROD
2682:to
2555:of
2271:PDB
2233:doi
2198:doi
2157:doi
2126:PMC
2118:doi
2083:PMC
2075:doi
2042:doi
2038:200
2011:doi
1976:PMC
1943:doi
1939:102
1914:doi
1910:138
1883:doi
1879:104
1850:doi
1838:234
1811:PMC
1803:doi
1762:doi
1731:doi
1727:261
1696:doi
1661:PMC
1653:doi
1612:doi
1573:hdl
1565:doi
1532:doi
1528:299
1490:PMC
1480:doi
1468:105
1433:doi
1429:109
1396:PMC
1388:doi
1140:PDB
431:End
330:End
233:OMA
221:320
209:MGI
57:PDB
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2961::
2911::
2800::
2789:EC
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