379:
295:
2975:
331:, and so on) produce NAG through ornithine acetyltransferase (OAT), which is part of a ‘cyclic’ ornithine production pathway. NAGS is therefore used in a supportive role, replenishing NAG reserves as required. In some plants and bacteria, however, NAGS catalyzes the first step in a ‘linear’ arginine production pathway.
32:
334:
The protein sequences of NAGS between prokaryotes, lower eukaryotes and higher eukaryotes have shown a remarkable lack of similarity. Sequence identity between prokaryotic and eukaryotic NAGS is largely <30%, while sequence identity between lower and higher eukaryotes is ~20%.
423:
suggest that NAGS proceeds through the previously described one-step mechanism. In this proposal, the carbonyl group of acetyl-CoA is attacked directly by the α-amino nitrogen of glutamate. This mechanism is supported by the activation of the carbonyl through
369:
is plentiful within the cell, and needs to be removed, accelerating NAGS function. As it stands, the evolutionary journey of NAGS from essential synthetic enzyme to primary urea cycle controller is yet to be fully understood.
462:
has shown promise as a possible treatment for NAGS deficiency. This is suspected to be a result of the structural similarities between NAG and carbamoyl glutamate, which allows carbamoyl glutamate to act as an effective
454:-acetylglutamate is an essential allosteric cofactor of CPS1, the enzyme that catalyzes the first step of the urea cycle. Without NAG stimulation, CPS1 cannot convert ammonia to
1507:
353:. While the role of arginine as an inhibitor of NAG in ornithine and arginine synthesis is well understood, there is some controversy as to the role of NAG in the
61:
2511:
2506:
1371:
2312:
1988:
357:. The currently accepted role of NAG in vertebrates is as an essential allosteric cofactor for CPS1, and therefore it acts as the primary controller of
2494:
1983:
2074:
1656:
2501:
1742:
2997:
440:
1551:
2489:
1500:
2661:
935:
Wong LJ, Wong SS (September 1983). "Kinetic mechanism of the reaction catalyzed by nuclear histone acetyltransferase from calf thymus".
85:
2629:
1842:
1777:
2694:
2445:
2383:
1787:
1612:
2440:
2435:
2113:
1911:
1546:
1415:
1364:
73:
66:
3012:
2378:
2280:
2108:
1772:
1493:
431:, as well as the absence of a suitable cysteine within the active site to act as an intermediate acceptor of the acetyl group.
1288:
Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M (October 2004). "Restoration of ureagenesis in
2393:
2305:
2103:
1889:
1847:
2850:
378:
2558:
2538:
2452:
2965:
2479:
2373:
2368:
1693:
1635:
1525:
276:
2641:
2415:
1747:
1357:
294:
2651:
2484:
1916:
1720:
1586:
1439:
1420:
398:-acetyltransferase function have been proposed: a two-step, ping-pong mechanism involving transfer of the relevant
2835:
2951:
2938:
2925:
2912:
2899:
2886:
2873:
2599:
2575:
2526:
2410:
2363:
2338:
2298:
1646:
2845:
2799:
2742:
2604:
2329:
1837:
1715:
1343:
256:, although its role and structure differ widely depending on the species. NAG can be used in the production of
126:
2747:
2646:
2553:
1884:
1671:
1444:
272:
2614:
2609:
2474:
1879:
1536:
78:
1349:
459:
2768:
2687:
2594:
2589:
2253:
1993:
1869:
1705:
1683:
1474:
1196:
Caldovic L, Morizono H, Panglao MG, Cheng SF, Packman S, Tuchman M (April 2003). "Null mutations in the
737:-glutamate synthetase from rat liver mitochondria and substrate and activator specificity of the enzyme"
419:
143:
2840:
1339:
2656:
2543:
2530:
2430:
2094:
2000:
1857:
1806:
1752:
1406:
288:
3002:
2804:
2388:
455:
362:
242:
2737:
2024:
1792:
1666:
1384:
1225:
603:
391:
3007:
2619:
2348:
2014:
1817:
1598:
1313:
1270:
1217:
1175:
1119:
1064:
1005:
952:
917:
864:
811:
758:
709:
656:
595:
556:
507:
204:
147:
2783:
2778:
2752:
2680:
2584:
2548:
1827:
1620:
1305:
1260:
1209:
1165:
1157:
1109:
1099:
1054:
1044:
995:
987:
944:
907:
899:
854:
846:
801:
793:
748:
699:
691:
646:
638:
587:
546:
538:
497:
346:
138:
1200:-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia".
2830:
2814:
2727:
2624:
2325:
2290:
1732:
1625:
1023:
Shi D, Sagar V, Jin Z, Yu X, Caldovic L, Morizono H, Allewell NM, Tuchman M (March 2008).
284:
167:
131:
2979:
2868:
2809:
1170:
1141:
1114:
1083:
1059:
1024:
1000:
971:
912:
883:
859:
830:
806:
777:
651:
622:
591:
502:
485:
447:
428:
753:
728:
704:
675:
551:
526:
2991:
2773:
2732:
1516:
1082:
Min L, Jin Z, Caldovic L, Morizono H, Allewell NM, Tuchman M, Shi D (February 2009).
425:
1229:
991:
607:
2722:
2149:
2124:
1782:
1661:
1574:
542:
399:
695:
102:
2946:
2881:
2717:
2563:
2321:
1309:
1161:
642:
350:
324:
265:
249:
177:
2974:
1901:
1563:
1388:
1380:
1334:
1213:
354:
339:
269:
253:
215:
109:
1265:
1244:
2920:
2894:
1832:
257:
211:
1317:
1221:
1179:
1123:
1104:
1068:
1049:
1009:
921:
660:
574:
Yu YG, Turner GE, Weiss RL (November 1996). "Acetylglutamate synthase from
1274:
956:
868:
815:
762:
599:
560:
511:
2353:
1978:
1852:
1485:
1449:
713:
414:
410:
403:
316:
261:
114:
948:
2138:
2133:
2053:
1430:
903:
464:
366:
850:
797:
621:
Caldovic L, Ah Mew N, Shi D, Morizono H, Yudkoff M, Tuchman M (2010).
2933:
2703:
2634:
2462:
2457:
2398:
2243:
2238:
2208:
2203:
2083:
2063:
2058:
2048:
2043:
2038:
2033:
1391:
328:
320:
200:
97:
2907:
2516:
2403:
2263:
2258:
2248:
2233:
2228:
2223:
2218:
2213:
2198:
2193:
2188:
2183:
2178:
2173:
2168:
2163:
2158:
1954:
1941:
676:"Organization and control in the arginine biosynthetic pathway of
407:
377:
293:
280:
2420:
358:
90:
2676:
2294:
1489:
1353:
31:
1335:
GeneReviews/NCBI/NIH/UW entry on Urea Cycle
Disorders Overview
1033:
provides insights into mechanisms of catalysis and regulation"
406:
residue and a one-step mechanism through direct attack of the
525:
Cunin R, Glansdorff N, Piérard A, Stalon V (September 1986).
835:-acetylglutamate a short-term regulator of urea synthesis?"
627:-Acetylglutamate synthase: structure, function and defects"
2672:
888:-Acetylglutamate and its changing role through evolution"
1140:
Morizono H, Caldovic L, Shi D, Tuchman M (April 2004).
484:
Meijer AJ, Lof C, Ramos IC, Verhoeven AJ (April 1985).
279:(CPS1). In mammals, NAGS is expressed primarily in the
980:
Annual Review of
Biophysics and Biomolecular Structure
2963:
1249:-acetylglutamate on carbamoyl-phosphate synthetase I"
527:"Biosynthesis and metabolism of arginine in bacteria"
2859:
2823:
2792:
2761:
2710:
2574:
2525:
2337:
2147:
2122:
2092:
2072:
2022:
2013:
1967:
1930:
1898:
1866:
1814:
1805:
1761:
1729:
1702:
1680:
1643:
1634:
1611:
1583:
1560:
1533:
1524:
1462:
1429:
1405:
1398:
173:
163:
158:
137:
125:
120:
108:
96:
84:
72:
60:
52:
47:
21:
221:Put simply NAGS catalyzes the following reaction:
417:group. Studies conducted using NAGS derived from
1191:
1189:
1135:
1133:
349:in plant and bacterial NAGS, but an effector in
2688:
2306:
1501:
1365:
8:
2512:2-acylglycerol-3-phosphate O-acyltransferase
2507:1-acylglycerol-3-phosphate O-acyltransferase
365:from arginine would act to signal NAGS that
1088:-acetyl-L-glutamate synthase by L-arginine"
976:-acetyltransferases: a structural overview"
578:: structure and regulation of expression".
458:, resulting in toxic ammonia accumulation.
39:-Acetylglutamate synthase/kinase tetramer,
2695:
2681:
2673:
2313:
2299:
2291:
2019:
1989:Mitochondrial permeability transition pore
1971:
1811:
1640:
1530:
1508:
1494:
1486:
1402:
1372:
1358:
1350:
155:
30:
1342:at the U.S. National Library of Medicine
1264:
1169:
1113:
1103:
1058:
1048:
999:
911:
858:
805:
752:
703:
650:
550:
501:
1984:Mitochondrial membrane transport protein
1292:-acetylglutamate synthase deficiency by
1243:McCudden CR, Powers-Lee SG (July 1996).
776:Meijer AJ, Verhoeven AJ (October 1984).
338:Enzyme activity of NAGS is modulated by
2970:
1245:"Required allosteric effector site for
1084:"Mechanism of allosteric inhibition of
476:
2502:Glycerol-3-phosphate O-acyltransferase
1743:Cholesterol side-chain cleavage enzyme
361:through the urea cycle. In this role,
248:family of enzymes, is present in both
18:
2495:Lecithin—cholesterol acyltransferase
970:Dyda F, Klein DC, Hickman AB (2000).
302:-acetylglutamate (NAG) synthesis via
7:
2490:Glyceronephosphate O-acyltransferase
782:-Acetylglutamate and urea synthesis"
727:Sonoda T, Tatibana M (August 1983).
444:-acetylglutamate synthase deficiency
382:A simplified reaction mechanism for
2662:Sulfoacetaldehyde acetyltransferase
2354:Acetyl-Coenzyme A acetyltransferase
1657:Coenzyme Q – cytochrome c reductase
1253:The Journal of Biological Chemistry
1092:The Journal of Biological Chemistry
1037:The Journal of Biological Chemistry
882:Caldovic L, Tuchman M (June 2003).
741:The Journal of Biological Chemistry
2578:: converted into alkyl on transfer
1843:Oxoglutarate dehydrogenase complex
1778:Glycerol-3-phosphate dehydrogenase
1029:-acetyl-L-glutamate synthase from
592:10.1046/j.1365-2958.1996.1321494.x
503:10.1111/j.1432-1033.1985.tb08824.x
14:
2384:Chloramphenicol acetyltransferase
1788:Carnitine palmitoyltransferase II
1150:Molecular Genetics and Metabolism
631:Molecular Genetics and Metabolism
203:that catalyses the production of
2973:
2436:Carnitine O-palmitoyltransferase
1912:Carbamoyl phosphate synthetase I
1552:Long-chain-fatty-acid—CoA ligase
1547:Carnitine palmitoyltransferase I
1416:Carbamoyl phosphate synthetase I
829:Lund P, Wiggins D (March 1984).
490:European Journal of Biochemistry
386:-acetylglutamate synthase (NAGS)
306:-acetylglutamate synthase (NAGS)
2379:Beta-galactoside transacetylase
1773:Glutamate aspartate transporter
992:10.1146/annurev.biophys.29.1.81
674:Cybis J, Davis RH (July 1975).
2394:Serotonin N-acetyl transferase
2341:: other than amino-acyl groups
1890:Pyruvate dehydrogenase complex
1848:Succinyl coenzyme A synthetase
543:10.1128/MMBR.50.3.314-352.1986
439:Inactivity of NAGS results in
1:
2559:Keratinocyte transglutaminase
2539:Gamma-glutamyl transpeptidase
2453:Serine C-palmitoyltransferase
754:10.1016/S0021-9258(17)44574-1
696:10.1128/JB.123.1.196-202.1975
2998:Genes on human chromosome 17
2480:Aminolevulinic acid synthase
2374:Acetyl-CoA C-acyltransferase
2369:Dihydrolipoyl transacetylase
1694:Dihydroorotate dehydrogenase
1440:Argininosuccinate synthetase
298:Overall reaction scheme for
277:carbamoyl phosphate synthase
2642:2-hydroxyglutarate synthase
1748:Steroid 11-beta-hydroxylase
1310:10.1016/j.jpeds.2004.06.047
1162:10.1016/j.ymgme.2003.10.017
643:10.1016/j.ymgme.2010.02.018
3029:
2652:2-isopropylmalate synthase
2485:Beta-ketoacyl-ACP synthase
2359:N-Acetylglutamate synthase
1922:N-Acetylglutamate synthase
1917:Ornithine transcarbamylase
1721:Glycerol phosphate shuttle
1587:monoamine neurotransmitter
1470:N-Acetylglutamate synthase
1421:Ornithine transcarbamylase
1340:N-Acetylglutamate+Synthase
1146:-acetylglutamate synthase"
1025:"The crystal structure of
287:, and is localized to the
2851:Michaelis–Menten kinetics
2600:Decylhomocitrate synthase
2411:Histone acetyltransferase
2364:Choline acetyltransferase
2276:
1974:
1950:
1647:oxidative phosphorylation
1298:The Journal of Pediatrics
1214:10.1007/s00439-003-0909-5
193:-Acetylglutamate synthase
154:
29:
25:-Acetylglutamate synthase
2743:Diffusion-limited enzyme
2605:2-methylcitrate synthase
1838:Isocitrate dehydrogenase
1716:Malate-aspartate shuttle
1344:Medical Subject Headings
1266:10.1074/jbc.271.30.18285
486:"Control of ureogenesis"
319:) and lower eukaryotes (
2647:3-propylmalate synthase
2554:Tissue transglutaminase
1885:Glutamate dehydrogenase
1672:Succinate dehydrogenase
1445:Argininosuccinate lyase
892:The Biochemical Journal
839:The Biochemical Journal
786:The Biochemical Journal
684:Journal of Bacteriology
531:Microbiological Reviews
450:. In many vertebrates,
3013:Mitochondrial proteins
2615:3-ethylmalate synthase
2610:2-ethylmalate synthase
2475:Acyltransferase like 2
2281:mitochondrial diseases
1880:Aspartate transaminase
1537:fatty acid degradation
1105:10.1074/jbc.M805348200
1050:10.1074/jbc.M707678200
580:Molecular Microbiology
387:
307:
241:NAGS, a member of the
2836:Eadie–Hofstee diagram
2769:Allosteric regulation
2595:Citrate (Re)-synthase
2590:Decylcitrate synthase
2531:Aminoacyltransferases
2431:palmitoyltransferases
1994:Mitochondrial carrier
1870:anaplerotic reactions
1706:mitochondrial shuttle
1684:pyrimidine metabolism
1475:Ornithine translocase
1385:amino acid metabolism
1296:-carbamylglutamate".
1031:Neisseria gonorrhoeae
435:Clinical significance
420:Neisseria gonorrhoeae
381:
297:
2846:Lineweaver–Burk plot
2657:Homocitrate synthase
2544:Peptidyl transferase
2001:Translocator protein
1858:Malate dehydrogenase
1753:Aldosterone synthase
1463:Regulatory/transport
1407:mitochondrial matrix
289:mitochondrial matrix
2389:N-acetyltransferase
1613:Intermembrane space
949:10.1021/bi00289a004
460:Carbamoyl glutamate
456:carbamoyl phosphate
363:feedback regulation
345:, which acts as an
311:Biological function
229:-glutamate → CoA +
2805:Enzyme superfamily
2738:Enzyme promiscuity
2349:acetyltransferases
1968:Other/to be sorted
1933:alcohol metabolism
1793:Uncoupling protein
1667:NADH dehydrogenase
1156:(Suppl 1): S4–11.
904:10.1042/BJ20030002
637:(Suppl 1): S13–9.
388:
315:Most prokaryotes (
308:
246:-acetyltransferase
2961:
2960:
2670:
2669:
2620:ATP citrate lyase
2288:
2287:
2272:
2271:
2015:Mitochondrial DNA
2009:
2008:
1963:
1962:
1818:citric acid cycle
1801:
1800:
1607:
1606:
1599:Monoamine oxidase
1483:
1482:
1458:
1457:
851:10.1042/bj2180991
798:10.1042/bj2230559
729:"Purification of
576:Neurospora crassa
342:
236:
228:
187:
186:
183:
182:
3020:
2978:
2977:
2969:
2841:Hanes–Woolf plot
2784:Enzyme activator
2779:Enzyme inhibitor
2753:Enzyme catalysis
2697:
2690:
2683:
2674:
2630:HMG-CoA synthase
2585:Citrate synthase
2549:Transglutaminase
2326:acyltransferases
2315:
2308:
2301:
2292:
2152:
2127:
2097:
2077:
2027:
2020:
1972:
1935:
1905:
1873:
1828:Citrate synthase
1821:
1812:
1766:
1736:
1709:
1687:
1650:
1641:
1621:Adenylate kinase
1592:
1568:
1540:
1531:
1510:
1503:
1496:
1487:
1403:
1374:
1367:
1360:
1351:
1322:
1321:
1285:
1279:
1278:
1268:
1259:(30): 18285–94.
1240:
1234:
1233:
1193:
1184:
1183:
1173:
1137:
1128:
1127:
1117:
1107:
1079:
1073:
1072:
1062:
1052:
1020:
1014:
1013:
1003:
967:
961:
960:
932:
926:
925:
915:
898:(Pt 2): 279–90.
879:
873:
872:
862:
826:
820:
819:
809:
773:
767:
766:
756:
724:
718:
717:
707:
671:
665:
664:
654:
618:
612:
611:
571:
565:
564:
554:
522:
516:
515:
505:
481:
402:to an activated
340:
264:, two important
234:
226:
208:-acetylglutamate
156:
41:Maricaulis maris
34:
19:
16:Class of enzymes
3028:
3027:
3023:
3022:
3021:
3019:
3018:
3017:
2988:
2987:
2984:
2972:
2964:
2962:
2957:
2869:Oxidoreductases
2855:
2831:Enzyme kinetics
2819:
2815:List of enzymes
2788:
2757:
2728:Catalytic triad
2706:
2701:
2671:
2666:
2625:Malate synthase
2570:
2521:
2333:
2319:
2289:
2284:
2268:
2148:
2143:
2123:
2118:
2093:
2088:
2073:
2068:
2023:
2005:
1959:
1946:
1931:
1926:
1899:
1894:
1867:
1862:
1815:
1797:
1762:
1757:
1733:steroidogenesis
1730:
1725:
1703:
1698:
1681:
1676:
1644:
1630:
1626:Creatine kinase
1603:
1589:
1584:
1579:
1561:
1556:
1534:
1520:
1514:
1484:
1479:
1454:
1425:
1394:
1378:
1331:
1326:
1325:
1287:
1286:
1282:
1242:
1241:
1237:
1195:
1194:
1187:
1139:
1138:
1131:
1081:
1080:
1076:
1043:(11): 7176–84.
1022:
1021:
1017:
969:
968:
964:
943:(20): 4637–41.
934:
933:
929:
881:
880:
876:
828:
827:
823:
775:
774:
770:
747:(16): 9839–44.
736:
726:
725:
721:
673:
672:
668:
620:
619:
615:
573:
572:
568:
524:
523:
519:
483:
482:
478:
473:
437:
376:
313:
285:small intestine
43:
17:
12:
11:
5:
3026:
3024:
3016:
3015:
3010:
3005:
3000:
2990:
2989:
2983:
2982:
2959:
2958:
2956:
2955:
2942:
2929:
2916:
2903:
2890:
2877:
2863:
2861:
2857:
2856:
2854:
2853:
2848:
2843:
2838:
2833:
2827:
2825:
2821:
2820:
2818:
2817:
2812:
2807:
2802:
2796:
2794:
2793:Classification
2790:
2789:
2787:
2786:
2781:
2776:
2771:
2765:
2763:
2759:
2758:
2756:
2755:
2750:
2745:
2740:
2735:
2730:
2725:
2720:
2714:
2712:
2708:
2707:
2702:
2700:
2699:
2692:
2685:
2677:
2668:
2667:
2665:
2664:
2659:
2654:
2649:
2644:
2639:
2638:
2637:
2627:
2622:
2617:
2612:
2607:
2602:
2597:
2592:
2587:
2581:
2579:
2572:
2571:
2569:
2568:
2567:
2566:
2561:
2556:
2546:
2541:
2535:
2533:
2523:
2522:
2520:
2519:
2514:
2509:
2504:
2498:
2497:
2492:
2487:
2482:
2477:
2468:
2467:
2466:
2465:
2460:
2450:
2449:
2448:
2443:
2426:
2425:
2424:
2423:
2418:
2408:
2407:
2406:
2401:
2396:
2386:
2381:
2376:
2371:
2366:
2361:
2356:
2344:
2342:
2335:
2334:
2320:
2318:
2317:
2310:
2303:
2295:
2286:
2285:
2277:
2274:
2273:
2270:
2269:
2267:
2266:
2261:
2256:
2251:
2246:
2241:
2236:
2231:
2226:
2221:
2216:
2211:
2206:
2201:
2196:
2191:
2186:
2181:
2176:
2171:
2166:
2161:
2155:
2153:
2145:
2144:
2142:
2141:
2136:
2130:
2128:
2120:
2119:
2117:
2116:
2111:
2106:
2100:
2098:
2090:
2089:
2087:
2086:
2080:
2078:
2070:
2069:
2067:
2066:
2061:
2056:
2051:
2046:
2041:
2036:
2030:
2028:
2017:
2011:
2010:
2007:
2006:
2004:
2003:
1998:
1997:
1996:
1991:
1981:
1975:
1969:
1965:
1964:
1961:
1960:
1958:
1957:
1951:
1948:
1947:
1945:
1944:
1938:
1936:
1928:
1927:
1925:
1924:
1919:
1914:
1908:
1906:
1896:
1895:
1893:
1892:
1887:
1882:
1876:
1874:
1864:
1863:
1861:
1860:
1855:
1850:
1845:
1840:
1835:
1830:
1824:
1822:
1809:
1803:
1802:
1799:
1798:
1796:
1795:
1790:
1785:
1780:
1775:
1769:
1767:
1759:
1758:
1756:
1755:
1750:
1745:
1739:
1737:
1727:
1726:
1724:
1723:
1718:
1712:
1710:
1700:
1699:
1697:
1696:
1690:
1688:
1678:
1677:
1675:
1674:
1669:
1664:
1659:
1653:
1651:
1638:
1636:Inner membrane
1632:
1631:
1629:
1628:
1623:
1617:
1615:
1609:
1608:
1605:
1604:
1602:
1601:
1595:
1593:
1581:
1580:
1578:
1577:
1571:
1569:
1558:
1557:
1555:
1554:
1549:
1543:
1541:
1528:
1526:Outer membrane
1522:
1521:
1515:
1513:
1512:
1505:
1498:
1490:
1481:
1480:
1478:
1477:
1472:
1466:
1464:
1460:
1459:
1456:
1455:
1453:
1452:
1447:
1442:
1436:
1434:
1427:
1426:
1424:
1423:
1418:
1412:
1410:
1400:
1396:
1395:
1379:
1377:
1376:
1369:
1362:
1354:
1348:
1347:
1337:
1330:
1329:External links
1327:
1324:
1323:
1280:
1235:
1202:Human Genetics
1185:
1129:
1098:(8): 4873–80.
1074:
1015:
972:"GCN5-related
962:
927:
874:
821:
768:
734:
719:
690:(1): 196–202.
666:
613:
566:
517:
475:
474:
472:
469:
448:hyperammonemia
436:
433:
375:
372:
312:
309:
239:
238:
185:
184:
181:
180:
175:
171:
170:
165:
161:
160:
152:
151:
141:
135:
134:
129:
123:
122:
118:
117:
112:
106:
105:
100:
94:
93:
88:
82:
81:
76:
70:
69:
64:
58:
57:
54:
50:
49:
45:
44:
35:
27:
26:
15:
13:
10:
9:
6:
4:
3:
2:
3025:
3014:
3011:
3009:
3006:
3004:
3001:
2999:
2996:
2995:
2993:
2986:
2981:
2976:
2971:
2967:
2953:
2949:
2948:
2943:
2940:
2936:
2935:
2930:
2927:
2923:
2922:
2917:
2914:
2910:
2909:
2904:
2901:
2897:
2896:
2891:
2888:
2884:
2883:
2878:
2875:
2871:
2870:
2865:
2864:
2862:
2858:
2852:
2849:
2847:
2844:
2842:
2839:
2837:
2834:
2832:
2829:
2828:
2826:
2822:
2816:
2813:
2811:
2810:Enzyme family
2808:
2806:
2803:
2801:
2798:
2797:
2795:
2791:
2785:
2782:
2780:
2777:
2775:
2774:Cooperativity
2772:
2770:
2767:
2766:
2764:
2760:
2754:
2751:
2749:
2746:
2744:
2741:
2739:
2736:
2734:
2733:Oxyanion hole
2731:
2729:
2726:
2724:
2721:
2719:
2716:
2715:
2713:
2709:
2705:
2698:
2693:
2691:
2686:
2684:
2679:
2678:
2675:
2663:
2660:
2658:
2655:
2653:
2650:
2648:
2645:
2643:
2640:
2636:
2633:
2632:
2631:
2628:
2626:
2623:
2621:
2618:
2616:
2613:
2611:
2608:
2606:
2603:
2601:
2598:
2596:
2593:
2591:
2588:
2586:
2583:
2582:
2580:
2577:
2573:
2565:
2562:
2560:
2557:
2555:
2552:
2551:
2550:
2547:
2545:
2542:
2540:
2537:
2536:
2534:
2532:
2528:
2524:
2518:
2515:
2513:
2510:
2508:
2505:
2503:
2500:
2499:
2496:
2493:
2491:
2488:
2486:
2483:
2481:
2478:
2476:
2473:
2470:
2469:
2464:
2461:
2459:
2456:
2455:
2454:
2451:
2447:
2444:
2442:
2439:
2438:
2437:
2434:
2432:
2428:
2427:
2422:
2419:
2417:
2414:
2413:
2412:
2409:
2405:
2402:
2400:
2397:
2395:
2392:
2391:
2390:
2387:
2385:
2382:
2380:
2377:
2375:
2372:
2370:
2367:
2365:
2362:
2360:
2357:
2355:
2352:
2350:
2346:
2345:
2343:
2340:
2336:
2331:
2327:
2323:
2316:
2311:
2309:
2304:
2302:
2297:
2296:
2293:
2283:
2282:
2275:
2265:
2262:
2260:
2257:
2255:
2252:
2250:
2247:
2245:
2242:
2240:
2237:
2235:
2232:
2230:
2227:
2225:
2222:
2220:
2217:
2215:
2212:
2210:
2207:
2205:
2202:
2200:
2197:
2195:
2192:
2190:
2187:
2185:
2182:
2180:
2177:
2175:
2172:
2170:
2167:
2165:
2162:
2160:
2157:
2156:
2154:
2151:
2146:
2140:
2137:
2135:
2132:
2131:
2129:
2126:
2121:
2115:
2112:
2110:
2107:
2105:
2102:
2101:
2099:
2096:
2091:
2085:
2082:
2081:
2079:
2076:
2071:
2065:
2062:
2060:
2057:
2055:
2052:
2050:
2047:
2045:
2042:
2040:
2037:
2035:
2032:
2031:
2029:
2026:
2021:
2018:
2016:
2012:
2002:
1999:
1995:
1992:
1990:
1987:
1986:
1985:
1982:
1980:
1977:
1976:
1973:
1970:
1966:
1956:
1953:
1952:
1949:
1943:
1940:
1939:
1937:
1934:
1929:
1923:
1920:
1918:
1915:
1913:
1910:
1909:
1907:
1904:
1903:
1897:
1891:
1888:
1886:
1883:
1881:
1878:
1877:
1875:
1872:
1871:
1865:
1859:
1856:
1854:
1851:
1849:
1846:
1844:
1841:
1839:
1836:
1834:
1831:
1829:
1826:
1825:
1823:
1820:
1819:
1813:
1810:
1808:
1804:
1794:
1791:
1789:
1786:
1784:
1781:
1779:
1776:
1774:
1771:
1770:
1768:
1765:
1760:
1754:
1751:
1749:
1746:
1744:
1741:
1740:
1738:
1735:
1734:
1728:
1722:
1719:
1717:
1714:
1713:
1711:
1708:
1707:
1701:
1695:
1692:
1691:
1689:
1686:
1685:
1679:
1673:
1670:
1668:
1665:
1663:
1660:
1658:
1655:
1654:
1652:
1649:
1648:
1642:
1639:
1637:
1633:
1627:
1624:
1622:
1619:
1618:
1616:
1614:
1610:
1600:
1597:
1596:
1594:
1591:
1588:
1582:
1576:
1573:
1572:
1570:
1567:
1565:
1559:
1553:
1550:
1548:
1545:
1544:
1542:
1539:
1538:
1532:
1529:
1527:
1523:
1518:
1517:Mitochondrial
1511:
1506:
1504:
1499:
1497:
1492:
1491:
1488:
1476:
1473:
1471:
1468:
1467:
1465:
1461:
1451:
1448:
1446:
1443:
1441:
1438:
1437:
1435:
1432:
1428:
1422:
1419:
1417:
1414:
1413:
1411:
1408:
1404:
1401:
1397:
1393:
1390:
1386:
1382:
1375:
1370:
1368:
1363:
1361:
1356:
1355:
1352:
1345:
1341:
1338:
1336:
1333:
1332:
1328:
1319:
1315:
1311:
1307:
1303:
1299:
1295:
1291:
1284:
1281:
1276:
1272:
1267:
1262:
1258:
1254:
1250:
1248:
1239:
1236:
1231:
1227:
1223:
1219:
1215:
1211:
1207:
1203:
1199:
1192:
1190:
1186:
1181:
1177:
1172:
1167:
1163:
1159:
1155:
1151:
1147:
1145:
1136:
1134:
1130:
1125:
1121:
1116:
1111:
1106:
1101:
1097:
1093:
1089:
1087:
1078:
1075:
1070:
1066:
1061:
1056:
1051:
1046:
1042:
1038:
1034:
1032:
1028:
1019:
1016:
1011:
1007:
1002:
997:
993:
989:
985:
981:
977:
975:
966:
963:
958:
954:
950:
946:
942:
938:
931:
928:
923:
919:
914:
909:
905:
901:
897:
893:
889:
887:
878:
875:
870:
866:
861:
856:
852:
848:
844:
840:
836:
834:
825:
822:
817:
813:
808:
803:
799:
795:
792:(2): 559–60.
791:
787:
783:
781:
772:
769:
764:
760:
755:
750:
746:
742:
738:
732:
723:
720:
715:
711:
706:
701:
697:
693:
689:
685:
681:
679:
670:
667:
662:
658:
653:
648:
644:
640:
636:
632:
628:
626:
617:
614:
609:
605:
601:
597:
593:
589:
586:(3): 545–54.
585:
581:
577:
570:
567:
562:
558:
553:
548:
544:
540:
537:(3): 314–52.
536:
532:
528:
521:
518:
513:
509:
504:
499:
496:(1): 189–96.
495:
491:
487:
480:
477:
470:
468:
466:
461:
457:
453:
449:
445:
443:
434:
432:
430:
427:
426:hydrogen bond
422:
421:
416:
412:
409:
405:
401:
397:
393:
385:
380:
373:
371:
368:
364:
360:
356:
352:
348:
344:
336:
332:
330:
326:
322:
318:
310:
305:
301:
296:
292:
290:
286:
282:
278:
274:
271:
267:
263:
259:
255:
251:
247:
245:
232:
225:acetyl-CoA +
224:
223:
222:
219:
217:
213:
209:
207:
202:
198:
194:
192:
179:
176:
172:
169:
166:
162:
157:
153:
150:
149:
145:
142:
140:
136:
133:
130:
128:
124:
119:
116:
113:
111:
107:
104:
101:
99:
95:
92:
89:
87:
83:
80:
77:
75:
71:
68:
65:
63:
59:
55:
51:
46:
42:
38:
33:
28:
24:
20:
2985:
2947:Translocases
2944:
2931:
2918:
2905:
2892:
2882:Transferases
2879:
2866:
2723:Binding site
2471:
2429:
2358:
2347:
2322:Transferases
2278:
2125:ATP synthase
1932:
1921:
1900:
1868:
1816:
1783:ATP synthase
1763:
1731:
1704:
1682:
1662:Cytochrome c
1645:
1585:
1575:Kynureninase
1562:
1535:
1469:
1304:(4): 552–4.
1301:
1297:
1293:
1289:
1283:
1256:
1252:
1246:
1238:
1208:(4): 364–8.
1205:
1201:
1197:
1153:
1149:
1143:
1095:
1091:
1085:
1077:
1040:
1036:
1030:
1026:
1018:
983:
979:
973:
965:
940:
937:Biochemistry
936:
930:
895:
891:
885:
877:
845:(3): 991–4.
842:
838:
832:
824:
789:
785:
779:
771:
744:
740:
730:
722:
687:
683:
677:
669:
634:
630:
624:
616:
583:
579:
575:
569:
534:
530:
520:
493:
489:
479:
451:
446:, a form of
441:
438:
429:polarization
418:
400:acetyl group
395:
389:
383:
337:
333:
314:
303:
299:
243:
240:
230:
220:
205:
196:
190:
189:
188:
146:
40:
36:
22:
2718:Active site
2564:Factor XIII
2075:Complex III
1142:"Mammalian
351:vertebrates
325:green algae
268:, or as an
266:amino acids
250:prokaryotes
210:(NAG) from
168:Swiss-model
48:Identifiers
3003:Urea cycle
2992:Categories
2921:Isomerases
2895:Hydrolases
2762:Regulation
2095:Complex IV
1902:urea cycle
1590:metabolism
1566:metabolism
1564:tryptophan
1399:Main cycle
1389:urea cycle
1381:Metabolism
986:: 81–103.
678:Neurospora
471:References
467:for CPS1.
392:mechanisms
355:urea cycle
270:allosteric
254:eukaryotes
237:-glutamate
216:acetyl-CoA
164:Structures
159:Search for
121:Other data
2800:EC number
2279:see also
2025:Complex I
1833:Aconitase
374:Mechanism
347:inhibitor
343:-arginine
258:ornithine
212:glutamate
127:EC number
103:NM_153006
62:NCBI gene
3008:EC 2.3.1
2824:Kinetics
2748:Cofactor
2711:Activity
2416:P300/CBP
1979:Frataxin
1853:Fumarase
1519:proteins
1450:Arginase
1318:15480384
1230:27479847
1222:12594532
1180:15050968
1124:19095660
1069:18184660
1010:10940244
922:12633501
733:-acetyl-
661:20303810
608:38149253
415:carbonyl
411:nitrogen
404:cysteine
317:bacteria
273:cofactor
262:arginine
233:-acetyl-
199:) is an
178:InterPro
2980:Biology
2934:Ligases
2704:Enzymes
2139:MT-ATP8
2134:MT-ATP6
2054:MT-ND4L
1431:cytosol
1392:enzymes
1275:8663466
1171:3031861
1115:2643497
1060:4099063
1001:4782277
957:6626521
913:1223426
869:6721845
860:1153434
816:6497864
807:1144333
763:6885773
652:2876818
600:8939437
561:3534538
512:3979393
465:agonist
413:on the
367:ammonia
174:Domains
144:Chr. 17
132:2.3.1.1
110:UniProt
2966:Portal
2908:Lyases
2635:HMGCS2
2472:other:
2463:SPTLC2
2458:SPTLC1
2399:HGSNAT
2244:MT-TS2
2239:MT-TS1
2209:MT-TL2
2204:MT-TL1
2114:MT-CO3
2109:MT-CO2
2104:MT-CO1
2084:MT-CYB
2064:MT-ND6
2059:MT-ND5
2049:MT-ND4
2044:MT-ND3
2039:MT-ND2
2034:MT-ND1
1807:Matrix
1346:(MeSH)
1316:
1273:
1228:
1220:
1178:
1168:
1122:
1112:
1067:
1057:
1008:
998:
955:
920:
910:
867:
857:
814:
804:
761:
714:166979
712:
705:235707
702:
659:
649:
606:
598:
559:
552:373073
549:
510:
329:plants
321:fungus
201:enzyme
148:q21.31
115:Q8N159
98:RefSeq
91:608300
67:162417
53:Symbol
2860:Types
2576:2.3.3
2527:2.3.2
2517:ABHD5
2404:ARD1A
2339:2.3.1
2264:MT-TY
2259:MT-TW
2254:MT-TV
2249:MT-TT
2234:MT-TR
2229:MT-TQ
2224:MT-TP
2219:MT-TN
2214:MT-TM
2199:MT-TK
2194:MT-TI
2189:MT-TH
2184:MT-TG
2179:MT-TF
2174:MT-TE
2169:MT-TD
2164:MT-TC
2159:MT-TA
1955:PMPCB
1942:ALDH2
1764:other
1226:S2CID
604:S2CID
408:amino
281:liver
139:Locus
79:17996
2952:list
2945:EC7
2939:list
2932:EC6
2926:list
2919:EC5
2913:list
2906:EC4
2900:list
2893:EC3
2887:list
2880:EC2
2874:list
2867:EC1
2446:CPT2
2441:CPT1
2421:NAT2
2332:2.3)
2150:tRNA
1314:PMID
1271:PMID
1218:PMID
1176:PMID
1120:PMID
1065:PMID
1006:PMID
953:PMID
918:PMID
865:PMID
831:"Is
812:PMID
759:PMID
710:PMID
657:PMID
596:PMID
557:PMID
508:PMID
394:for
390:Two
359:flux
283:and
275:for
260:and
252:and
214:and
197:NAGS
86:OMIM
74:HGNC
56:NAGS
1306:doi
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