247:
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121:
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2348:
2288:
2303:
2333:
2318:
505:
498:
29:
253:
152:
2391:
1127:
by western blot analysis. This result is consistent with a degradation of SUCLA2 when its heterodimer partner, SUCLG1, is absent. As mitochondrial DNA depletion in muscle is not a constant finding in SUCLG1 patients, diagnosis should not be based on it; additionally, it may be that alternative
1897:
Valayannopoulos V, Haudry C, Serre V, Barth M, Boddaert N, Arnoux JB, Cormier-Daire V, Rio M, Rabier D, Vassault A, Munnich A, Bonnefont JP, de Lonlay P, Rötig A, Lebre AS (Jun 2010). "New SUCLG1 patients expanding the phenotypic spectrum of this rare cause of mild methylmalonic aciduria".
1933:
Rouzier C, Le Guédard-Méreuze S, Fragaki K, Serre V, Miro J, Tuffery-Giraud S, Chaussenot A, Bannwarth S, Caruba C, Ostergaard E, Pellissier JF, Richelme C, Espil C, Chabrol B, Paquis-Flucklinger V (Oct 2010).
1606:
1603:
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1207:
1195:
1175:
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1601:
1205:
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1169:
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2347:
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2017:
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (Oct 1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library".
2302:
1055:
residue in the active site and the two alpha helices, one contributed by each subunit of the alphabeta-dimer to stabilize the structure. One of the alpha helices contains
2332:
2317:
260:
159:
2120:"A systematic analysis of human CHMP protein interactions: additional MIT domain-containing proteins bind to multiple components of the human ESCRT III complex"
2046:
Fraser ME, James MN, Bridger WA, Wolodko WT (Jun 2000). "Phosphorylated and dephosphorylated structures of pig heart, GTP-specific succinyl-CoA synthetase".
1816:
Fraser ME, James MN, Bridger WA, Wolodko WT (Jun 2000). "Phosphorylated and dephosphorylated structures of pig heart, GTP-specific succinyl-CoA synthetase".
758:
82:
739:
1568:
1172:
1583:
1187:
2460:
1988:
Maruyama K, Sugano S (Jan 1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides".
2436:
2267:
1743:
1725:
2455:
1599:
1586:
1572:
1203:
1190:
1176:
2155:"Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion"
1602:
1206:
246:
958:
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223:
1935:
1802:
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1213:
1185:
1160:
1712:
1691:
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1557:
1198:
1161:
1708:
1087:
and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile
145:
120:
1596:
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1200:
1165:
1687:
1612:
1611:
1570:
1558:
1216:
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62:
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1214:
1167:
259:
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1115:. Mutations in SUCLG1 lead to complete absence of SUCLG1 protein and are responsible for a very severe disorder with
1615:
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1202:
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1936:"The severity of phenotype linked to SUCLG1 mutations could be correlated with residual amount of SUCLG1 protein"
1618:
1617:
1608:
1578:
1573:
1222:
1221:
1212:
1182:
1177:
53:, GALPHA, MTDPS9, SUCLA1, succinate-CoA ligase alpha subunit, succinate-CoA ligase GDP/ADP-forming subunit alpha
1614:
1600:
1582:
1575:
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1562:
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1186:
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803:
70:
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1178:
1593:
1590:
1569:
1197:
1194:
1173:
784:
2371:: Crystal structure of pig GTP-specific succinyl-CoA synthetase from polyethylene glycol with chloride ions
2077:"Sequence comparison of human and mouse genes reveals a homologous block structure in the promoter regions"
2075:
Suzuki Y, Yamashita R, Shirota M, Sakakibara Y, Chiba J, Mizushima-Sugano J, Nakai K, Sugano S (Sep 2004).
1637:
1112:
2422:
2253:
134:
49:
2153:
Ostergaard E, Christensen E, Kristensen E, Mogensen B, Duno M, Shoubridge EA, Wibrand F (Aug 2007).
1966:
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94:
941:
920:
894:
873:
1059:, the modification of which result in conformational changes that accommodate either the bound
2225:
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2063:
2034:
2005:
1958:
1915:
1868:
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504:
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339:
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90:
1763:"The molecular basis for cross-reaction of an anti-dystrophin antibody with alpha-actinin"
1088:
1040:
314:
2356:: Crystal structure of pig GTP-specific succinyl-CoA synthetase from polyethylene glycol
28:
2296:: CRYSTAL STRUCTURE OF DEPHOSPHORYLATED PIG HEART, GTP-SPECIFIC SUCCINYL-COA SYNTHETASE
2179:
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2311:: CRYSTAL STRUCTURE OF PHOSPHORYLATED PIG HEART, GTP-SPECIFIC SUCCINYL-COA SYNTHETASE
2234:
2213:
2001:
1658:
1641:
1628:
1080:
1076:
1072:
1048:
533:
1970:
1880:
74:
2402:
2341:: Crystal structure of pig GTP-specific succinyl-CoA synthetase in complex with GDP
2326:: Crystal structure of pig GTP-specific succinyl-CoA synthetase in complex with GTP
1851:
Ostergaard E (Apr 2008). "Disorders caused by deficiency of succinate-CoA ligase".
1119:
manifestations. Furthermore, it is shown that in the absence of SUCLG1 protein, no
1084:
332:
111:
98:
2136:
2119:
1748:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1730:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1911:
415:
1864:
1141:
Click on genes, proteins and metabolites below to link to respective articles.
1124:
1056:
231:
128:
78:
2118:
Tsang HT, Connell JW, Brown SE, Thompson A, Reid E, Sanderson CM (Sep 2006).
1954:
1116:
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2110:
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2009:
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1005:
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989:
848:
829:
1128:
physiopathological mechanisms may be considered to explain the combined
556:
2239:
2229:
2218:
2208:
2092:
815:
770:
1803:"Entrez Gene: SUCLG1 succinate-CoA ligase, GDP-forming, alpha subunit"
1120:
1021:
973:
725:
2390:
2217:(Human Succinate--CoA ligase subunit alpha, mitochondrial) at the
2170:
2238:(Pig Succinate--CoA ligase subunit alpha, mitochondrial) at the
688:
684:
2398:
1767:
Biochimica et
Biophysica Acta (BBA) - Molecular Basis of Disease
1028:
66:
2249:
513:
2245:
2224:
Overview of all the structural information available in the
2203:
Overview of all the structural information available in the
1551:
1155:
1656:
The interactive pathway map can be edited at WikiPathways:
322:
2410:
487:
1761:James M, Man NT, Edwards YH, Morris GE (Apr 1997).
1103:Succinate-CoA ligase deficiency is responsible for
1039:The enzyme encoded by SUCLG1 can exist in either a
934:
913:
887:
866:
1704:
1702:
1700:
1683:
1681:
1679:
1079:coenzyme A ligase. This enzyme is targeted to the
1018:Succinyl-CoA ligase subunit alpha, mitochondrial
269:
168:
1555:
1159:
16:Protein-coding gene in the species Homo sapiens
1709:GRCm38: Ensembl release 89: ENSMUSG00000052738
2430:
2261:
8:
1892:
1890:
674:nucleoside triphosphate biosynthetic process
1688:GRCh38: Ensembl release 89: ENSG00000163541
1071:This gene encodes the alpha subunit of the
1047:form. In the dephosphorylated structure, a
572:succinate-CoA ligase (GDP-forming) activity
567:succinate-CoA ligase (ADP-forming) activity
2437:
2423:
2268:
2254:
2246:
699:
633:succinate-CoA ligase complex (GDP-forming)
529:
310:
209:
106:
2178:
2135:
2100:
1778:
2283:
1675:
1649:
1132:deficiency observed in these patients.
1853:Journal of Inherited Metabolic Disease
669:nucleoside diphosphate phosphorylation
577:nucleoside diphosphate kinase activity
18:
274:
235:
230:
173:
132:
127:
7:
2387:
2385:
2409:. You can help Knowledge (XXG) by
2159:American Journal of Human Genetics
931:
910:
884:
863:
839:
820:
794:
775:
749:
730:
492:
410:
348:
327:
14:
1063:group or the free phosphate ion.
1024:that in humans is encoded by the
2389:
2361:
2346:
2331:
2316:
2301:
2286:
1083:and catalyzes the conversion of
503:
496:
450:extensor digitorum longus muscle
258:
251:
245:
222:
157:
150:
144:
119:
27:
659:succinyl-CoA metabolic process
514:More reference expression data
476:More reference expression data
1:
2461:Human chromosome 2 gene stubs
2031:10.1016/S0378-1119(97)00411-3
1780:10.1016/s0925-4439(96)00076-2
1051:works in coordination with a
587:succinate-CoA ligase activity
243:
142:
2048:Journal of Molecular Biology
2002:10.1016/0378-1119(94)90802-8
1818:Journal of Molecular Biology
613:mitochondrial inner membrane
2456:Genes on human chromosome 2
2137:10.1016/j.ygeno.2006.04.003
1943:Journal of Medical Genetics
664:succinate metabolic process
2477:
2384:
1912:10.1016/j.mito.2010.02.006
454:sternocleidomastoid muscle
388:mucosa of transverse colon
2281:
1865:10.1007/s10545-008-0828-7
1744:"Mouse PubMed Reference:"
1726:"Human PubMed Reference:"
1004:
999:
995:
988:
972:
953:
938:
917:
906:
891:
870:
859:
846:
842:
827:
823:
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797:
782:
778:
769:
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752:
737:
733:
724:
709:
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682:
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528:
511:
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413:
360:
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191:
178:
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118:
109:
105:
60:
57:
47:
40:
35:
26:
21:
654:tricarboxylic acid cycle
1955:10.1136/jmg.2009.073445
1136:Interactive pathway map
966:Chr 6: 73.23 – 73.25 Mb
959:Chr 2: 84.42 – 84.46 Mb
462:vastus lateralis muscle
434:myocardium of ventricle
426:interventricular septum
396:mucosa of sigmoid colon
2060:10.1006/jmbi.2000.3807
1830:10.1006/jmbi.2000.3807
1621:
1225:
1113:methylmalonic aciduria
372:metanephric glomerulus
1620:
1224:
1099:Clinical significance
618:extracellular exosome
1627:|alt=TCACycle_WP78
1123:protein is found in
628:mitochondrial matrix
438:brown adipose tissue
237:Chromosome 6 (mouse)
135:Chromosome 2 (human)
1111:depletion and mild
2397:This article on a
2093:10.1101/gr.2435604
1622:
1226:
804:ENSMUSG00000052738
642:Biological process
596:Cellular component
557:catalytic activity
547:nucleotide binding
540:Molecular function
458:extraocular muscle
99:SUCLG1 - orthologs
2418:
2417:
2379:
2378:
1130:respiratory chain
1109:mitochondrial DNA
1105:encephalomyopathy
1093:mitochondrial DNA
1015:
1014:
1011:
1010:
984:
983:
949:
948:
928:
927:
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901:
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835:
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2013:
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1282:
1277:
1272:
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1262:
1257:
1252:
1247:
1242:
1237:
1232:
1045:dephosphorylated
997:
996:
968:
961:
944:
932:
923:
911:
907:RefSeq (protein)
897:
885:
876:
864:
840:
821:
795:
776:
750:
731:
700:
530:
516:
507:
500:
493:
478:
442:plantaris muscle
430:digastric muscle
418:
416:Top expressed in
411:
356:
354:Top expressed in
349:
328:
311:
301:
288:
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262:
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52:
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2195:
2152:
2117:
2081:Genome Research
2074:
2045:
2025:(1–2): 149–56.
2016:
1987:
1983:
1981:Further reading
1978:
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1666:
1659:"TCACycle_WP78"
1657:
1655:
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1640:TCACycle_WP78
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1101:
1089:lactic acidosis
1069:
1037:
1006:View/Edit Mouse
1001:View/Edit Human
964:
957:
954:Location (UCSC)
940:
919:
893:
872:
785:ENSG00000163541
678:
637:
603:plasma membrane
591:
552:ligase activity
512:
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392:right ventricle
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213:Gene location (
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110:Gene location (
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2199:
2198:External links
2196:
2194:
2193:
2171:10.1086/519222
2150:
2115:
2072:
2054:(5): 1325–39.
2043:
2014:
1996:(1–2): 171–4.
1984:
1982:
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1977:
1976:
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1886:
1843:
1824:(5): 1325–39.
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1149:
1148:
1147:
1146:
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1137:
1134:
1100:
1097:
1068:
1065:
1041:phosphorylated
1036:
1033:
1013:
1012:
1009:
1008:
1003:
993:
992:
986:
985:
982:
981:
979:
977:
970:
969:
962:
955:
951:
950:
947:
946:
936:
935:
929:
926:
925:
915:
914:
908:
904:
903:
900:
899:
889:
888:
882:
879:
878:
868:
867:
861:
857:
856:
853:
852:
844:
843:
837:
834:
833:
825:
824:
818:
812:
811:
808:
807:
799:
798:
792:
789:
788:
780:
779:
773:
767:
766:
763:
762:
754:
753:
747:
744:
743:
735:
734:
728:
722:
721:
716:
711:
707:
706:
696:
695:
692:
691:
680:
679:
677:
676:
671:
666:
661:
656:
651:
645:
643:
639:
638:
636:
635:
630:
625:
620:
615:
610:
605:
599:
597:
593:
592:
590:
589:
584:
579:
574:
569:
564:
559:
554:
549:
543:
541:
537:
536:
526:
525:
522:
521:
518:
517:
509:
508:
490:
484:
483:
480:
479:
471:
470:
467:
466:
464:
463:
459:
455:
451:
447:
443:
439:
435:
431:
427:
423:
420:
419:
408:
405:
404:
402:
401:
397:
393:
389:
385:
381:
380:jejunal mucosa
377:
373:
369:
365:
361:
358:
357:
345:
344:
336:
325:
319:
318:
315:RNA expression
307:
306:
303:
302:
294:
290:
289:
281:
278:
273:
267:
266:
257:
250:
244:
240:
239:
234:
228:
227:
219:
218:
206:
205:
202:
201:
193:
189:
188:
180:
177:
172:
166:
165:
156:
149:
143:
139:
138:
131:
125:
124:
116:
115:
103:
102:
59:
55:
54:
46:
38:
37:
33:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
2473:
2462:
2459:
2457:
2454:
2453:
2451:
2440:
2435:
2433:
2428:
2426:
2421:
2420:
2414:
2412:
2408:
2404:
2400:
2395:
2392:
2388:
2383:
2370:
2364:
2359:
2355:
2349:
2344:
2340:
2334:
2329:
2325:
2319:
2314:
2310:
2304:
2299:
2295:
2289:
2284:
2280:
2271:
2266:
2264:
2259:
2257:
2252:
2251:
2248:
2241:
2237:
2236:
2231:
2227:
2223:
2220:
2216:
2215:
2210:
2206:
2202:
2201:
2197:
2190:
2186:
2181:
2176:
2172:
2168:
2164:
2160:
2156:
2151:
2147:
2143:
2138:
2133:
2130:(3): 333–46.
2129:
2125:
2121:
2116:
2112:
2108:
2103:
2098:
2094:
2090:
2087:(9): 1711–8.
2086:
2082:
2078:
2073:
2069:
2065:
2061:
2057:
2053:
2049:
2044:
2040:
2036:
2032:
2028:
2024:
2020:
2015:
2011:
2007:
2003:
1999:
1995:
1991:
1986:
1985:
1980:
1972:
1968:
1964:
1960:
1956:
1952:
1949:(10): 670–6.
1948:
1944:
1937:
1929:
1926:
1921:
1917:
1913:
1909:
1906:(4): 335–41.
1905:
1901:
1900:Mitochondrion
1893:
1891:
1887:
1882:
1878:
1874:
1870:
1866:
1862:
1858:
1854:
1847:
1844:
1839:
1835:
1831:
1827:
1823:
1819:
1812:
1809:
1804:
1798:
1795:
1790:
1786:
1781:
1776:
1773:(2): 169–76.
1772:
1768:
1764:
1757:
1754:
1749:
1745:
1739:
1736:
1731:
1727:
1721:
1718:
1714:
1710:
1705:
1703:
1701:
1697:
1693:
1689:
1684:
1682:
1680:
1676:
1670:
1660:
1653:
1650:
1643:
1638:
1630:
1554:
1158:
1143:
1142:
1135:
1133:
1131:
1126:
1122:
1118:
1114:
1110:
1106:
1098:
1096:
1094:
1090:
1086:
1082:
1078:
1074:
1073:heterodimeric
1066:
1064:
1062:
1058:
1054:
1050:
1049:phosphate ion
1046:
1042:
1034:
1032:
1030:
1027:
1023:
1019:
1007:
1002:
998:
994:
991:
987:
980:
978:
975:
971:
967:
963:
960:
956:
952:
945:
943:
937:
933:
930:
924:
922:
916:
912:
909:
905:
898:
896:
890:
886:
883:
877:
875:
869:
865:
862:
860:RefSeq (mRNA)
858:
851:
850:
845:
841:
838:
832:
831:
826:
822:
819:
817:
813:
806:
805:
800:
796:
793:
787:
786:
781:
777:
774:
772:
768:
761:
760:
755:
751:
748:
742:
741:
736:
732:
729:
727:
723:
720:
717:
715:
712:
708:
705:
701:
697:
690:
686:
681:
675:
672:
670:
667:
665:
662:
660:
657:
655:
652:
650:
647:
646:
644:
641:
640:
634:
631:
629:
626:
624:
621:
619:
616:
614:
611:
609:
608:mitochondrion
606:
604:
601:
600:
598:
595:
594:
588:
585:
583:
580:
578:
575:
573:
570:
568:
565:
563:
560:
558:
555:
553:
550:
548:
545:
544:
542:
539:
538:
535:
534:Gene ontology
531:
527:
515:
510:
506:
499:
494:
491:
489:
485:
477:
472:
461:
457:
453:
449:
445:
441:
437:
433:
429:
425:
424:
421:
417:
412:
409:
400:apex of heart
399:
395:
391:
387:
383:
379:
376:renal medulla
375:
371:
367:
364:kidney tubule
363:
362:
359:
355:
350:
347:
346:
343:
341:
337:
335:
334:
330:
329:
326:
324:
320:
316:
312:
308:
300:
295:
291:
287:
282:
272:
268:
261:
254:
248:
241:
233:
229:
225:
220:
216:
211:
207:
199:
194:
190:
186:
181:
171:
167:
160:
153:
147:
140:
136:
130:
126:
122:
117:
113:
108:
104:
100:
96:
92:
88:
84:
80:
76:
72:
68:
64:
56:
51:
44:
39:
34:
30:
25:
20:
2411:expanding it
2403:chromosome 2
2396:
2381:
2368:
2353:
2338:
2323:
2308:
2293:
2233:
2212:
2165:(2): 383–7.
2162:
2158:
2127:
2123:
2084:
2080:
2051:
2047:
2022:
2018:
1993:
1989:
1946:
1942:
1928:
1903:
1899:
1859:(2): 226–9.
1856:
1852:
1846:
1821:
1817:
1811:
1797:
1770:
1766:
1756:
1747:
1738:
1729:
1720:
1652:
1140:
1139:
1102:
1085:succinyl CoA
1081:mitochondria
1070:
1038:
1025:
1017:
1016:
939:
918:
892:
871:
847:
828:
802:
783:
757:
738:
718:
713:
446:right kidney
338:
331:
58:External IDs
2276:PDB gallery
1125:fibroblasts
1095:depletion.
1057:amino acids
582:GDP binding
562:RNA binding
297:73,253,894
284:73,225,365
276:6|6 C1
196:84,460,045
183:84,423,528
36:Identifiers
2450:Categories
1715:, May 2017
1694:, May 2017
1671:References
1061:phosphoryl
1043:form or a
649:metabolism
368:glomerulus
342:(ortholog)
79:HomoloGene
2401:on human
1117:antenatal
1077:succinate
1053:histidine
1035:Structure
942:NP_063932
921:NP_003840
895:NM_019879
874:NM_003849
704:Orthologs
87:GeneCards
2189:17668387
2146:16730941
2124:Genomics
2111:15342556
2068:10873456
1971:35860287
1963:20693550
1920:20197121
1881:12722653
1873:18392745
1838:10873456
1711:–
1690:–
1067:Function
990:Wikidata
683:Sources:
384:duodenum
2240:PDBe-KB
2230:UniProt
2219:PDBe-KB
2209:UniProt
2180:1950792
2039:9373149
2010:8125298
1789:9128182
1713:Ensembl
1692:Ensembl
1150:[[File:
1075:enzyme
816:UniProt
771:Ensembl
710:Species
689:QuickGO
623:cytosol
317:pattern
75:1927234
43:Aliases
2235:O19069
2214:P53597
2187:
2177:
2144:
2109:
2102:515316
2099:
2066:
2037:
2008:
1969:
1961:
1918:
1879:
1871:
1836:
1787:
1121:SUCLA2
1026:SUCLG1
1022:enzyme
1020:is an
976:search
974:PubMed
849:Q9WUM5
830:P53597
726:Entrez
488:BioGPS
175:2p11.2
91:SUCLG1
67:611224
50:SUCLG1
22:SUCLG1
2405:is a
1967:S2CID
1939:(PDF)
1877:S2CID
1107:with
759:56451
719:Mouse
714:Human
685:Amigo
340:Mouse
333:Human
280:Start
215:Mouse
179:Start
112:Human
83:55785
2407:stub
2399:gene
2369:2fpp
2354:2fpi
2339:2fpg
2324:2fp4
2309:1eud
2294:1euc
2228:for
2207:for
2185:PMID
2142:PMID
2107:PMID
2064:PMID
2035:PMID
2019:Gene
2006:PMID
1990:Gene
1959:PMID
1916:PMID
1869:PMID
1834:PMID
1785:PMID
1771:1360
1642:edit
1629:edit
1091:and
1029:gene
740:8802
323:Bgee
271:Band
232:Chr.
170:Band
129:Chr.
63:OMIM
2226:PDB
2205:PDB
2175:PMC
2167:doi
2132:doi
2097:PMC
2089:doi
2056:doi
2052:299
2027:doi
2023:200
1998:doi
1994:138
1951:doi
1908:doi
1861:doi
1826:doi
1822:299
1775:doi
293:End
192:End
95:OMA
71:MGI
2452::
2232::
2211::
2183:.
2173:.
2163:81
2161:.
2157:.
2140:.
2128:88
2126:.
2122:.
2105:.
2095:.
2085:14
2083:.
2079:.
2062:.
2050:.
2033:.
2021:.
2004:.
1992:.
1965:.
1957:.
1947:47
1945:.
1941:.
1914:.
1904:10
1902:.
1889:^
1875:.
1867:.
1857:31
1855:.
1832:.
1820:.
1783:.
1769:.
1765:.
1746:.
1728:.
1699:^
1678:^
1631:]]
1031:.
687:/
299:bp
286:bp
198:bp
185:bp
93:;
89::
85:;
81::
77:;
73::
69:;
65::
2438:e
2431:t
2424:v
2413:.
2269:e
2262:t
2255:v
2242:.
2221:.
2191:.
2169::
2148:.
2134::
2113:.
2091::
2070:.
2058::
2041:.
2029::
2012:.
2000::
1973:.
1953::
1922:.
1910::
1883:.
1863::
1840:.
1828::
1805:.
1791:.
1777::
1750:.
1732:.
1661:.
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:]
1396:]
1391:]
1386:]
1381:]
1376:]
1371:]
1366:]
1361:]
1356:]
1351:]
1346:]
1341:]
1336:]
1331:]
1326:]
1321:]
1316:]
1311:]
1306:]
1301:]
1296:]
1291:]
1286:]
1281:]
1276:]
1271:]
1266:]
1261:]
1256:]
1251:]
1246:]
1241:]
1236:]
1231:]
217:)
114:)
97::
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