251:
228:
125:
150:
3283:
3298:
257:
156:
5133:
33:
1279:
formation of hamartomas and cancerous tumors. However, researchers are uncertain whether the identified SDHD gene variants are directly associated with Cowden syndrome and Cowden-like syndrome. Some of the variants described above have rarely been found in people without the features of these conditions.
2811:
Talmud PJ, Drenos F, Shah S, Shah T, Palmen J, Verzilli C, Gaunt TR, Pallas J, Lovering R, Li K, Casas JP, Sofat R, Kumari M, Rodriguez S, Johnson T, Newhouse SJ, Dominiczak A, Samani NJ, Caulfield M, Sever P, Stanton A, Shields DC, Padmanabhan S, Melander O, Hastie C, Delles C, Ebrahim S, Marmot MG,
2665:
Ricketts CJ, Forman JR, Rattenberry E, Bradshaw N, Lalloo F, Izatt L, Cole TR, Armstrong R, Kumar VK, Morrison PJ, Atkinson AB, Douglas F, Ball SG, Cook J, Srirangalingam U, Killick P, Kirby G, Aylwin S, Woodward ER, Evans DG, Hodgson SV, Murday V, Chew SL, Connell JM, Blundell TL, Macdonald F, Maher
3120:
Erlic Z, Rybicki L, Peczkowska M, Golcher H, Kann PH, Brauckhoff M, Müssig K, Muresan M, Schäffler A, Reisch N, Schott M, Fassnacht M, Opocher G, Klose S, Fottner C, Forrer F, Plöckinger U, Petersenn S, Zabolotny D, Kollukch O, Yaremchuk S, Januszewicz A, Walz MK, Eng C, Neumann HP (October 2009).
1257:
genes lead to the loss or reduction of SDH enzyme activity. Because the mutated SDH enzyme cannot convert succinate to fumarate, succinate accumulates in the cell. As a result, the hypoxia pathways are triggered in normal oxygen conditions, which lead to abnormal cell growth and tumor formation.
1182:
interactions with Tyr83 of SDHD. The presence of electrons in the iron sulphur cluster induces the movement of ubiquinone into a second orientation. This facilitates a second hydrogen bond interaction between the O4 carbonyl group of ubiquinone and Ser27 of subunit C. Following the first single
1278:
cancers. The SDHD gene variants associated with Cowden syndrome and Cowden-like syndrome change single amino acids in the SDHD protein, which likely alters the function of the SDH enzyme. Studies suggest that the defective enzyme could allow cells to grow and divide unchecked, leading to the
3158:
Krawczyk A, Hasse-Lazar K, Pawlaczek A, Szpak-Ulczok S, Krajewska J, Paliczka-Cieślak E, Jurecka-Lubieniecka B, Roskosz J, Chmielik E, Ziaja J, Cierpka L, Peczkowska M, Preibisz A, Januszewicz A, Otto M, Jarzab B (2010). "Germinal mutations of RET, SDHB, SDHD, and VHL genes in patients with
2006:
Hirawake H, Taniwaki M, Tamura A, Kojima S, Kita K (1997). "Cytochrome b in human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the components in liver mitochondria and chromosome assignment of the genes for the large (SDHC) and small (SDHD) subunits to 1q21 and 11q23".
2200:
Baysal BE, Ferrell RE, Willett-Brozick JE, Lawrence EC, Myssiorek D, Bosch A, van der Mey A, Taschner PE, Rubinstein WS, Myers EN, Richard CW, Cornelisse CJ, Devilee P, Devlin B (February 2000). "Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma".
2589:
CascĂłn A, LĂłpez-JimĂ©nez E, Landa I, Leskelä S, Leandro-GarcĂa LJ, Maliszewska A, LetĂłn R, de la Vega L, GarcĂa-Barcina MJ, Sanabria C, Alvarez-Escolá C, RodrĂguez-Antona C, Robledo M (September 2009). "Rationalization of genetic testing in patients with apparently sporadic
1313:
gene. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest
1970:
Heutink P, van der Mey AG, Sandkuijl LA, van Gils AP, Bardoel A, Breedveld GJ, van Vliet M, van Ommen GJ, Cornelisse CJ, Oostra BA (April 1992). "A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter".
2709:
Gill AJ, Benn DE, Chou A, Clarkson A, Muljono A, Meyer-Rochow GY, Richardson AL, Sidhu SB, Robinson BG, Clifton-Bligh RJ (June 2010). "Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes".
1302:(a type of paraganglioma). An inherited SDHD gene mutation predisposes an individual to cancer formation. An additional mutation that deletes the normal copy of the gene is needed to cause Carney-Stratakis syndrome. This second mutation, called a
2328:
Jackson CB, Nuoffer JM, Hahn D, Prokisch H, Haberberger B, Gautschi M, Häberli A, Gallati S, Schaller A (March 2014). "Mutations in SDHD lead to autosomal recessive encephalomyopathy and isolated mitochondrial complex II deficiency".
2992:
Richalet JP, Gimenez-Roqueplo AP, Peyrard S, VĂ©nisse A, Marelle L, Burnichon N, Bouzamondo A, Jeunemaitre X, Azizi M, Elghozi JL (December 2009). "A role for succinate dehydrogenase genes in low chemoresponsiveness to hypoxia?".
2042:
Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P (October 2013).
4624:
2627:
Waldmann J, Langer P, Habbe N, Fendrich V, Ramaswamy A, Rothmund M, Bartsch DK, Slater EP (June 2009). "Mutations and polymorphisms in the SDHB, SDHD, VHL, and RET genes in sporadic and familial pheochromocytomas".
1269:
and an increased risk of developing certain cancers. When Cowden syndrome and Cowden-like syndrome are caused by SDHD gene mutations, the conditions are associated with a particularly high risk of developing
1796:
1793:
1781:
1761:
1757:
1400:
1397:
1385:
1365:
1361:
1791:
1395:
3282:
2161:"Structural and computational analysis of the quinone-binding site of complex II (succinate-ubiquinone oxidoreductase): a mechanism of electron transfer and proton conduction during ubiquinone reduction"
1258:
People living at higher altitudes (for example, the Andes mountains) are known to have an increased rate of benign paraganglioma, with the rate of disease increasing with the altitude of the population.
3714:
3338:
1743:
1347:
1309:
Mitochondrial complex II deficiency (MT-C2D), a disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, has also been associated with mutations in the
3297:
1794:
1785:
1755:
1754:
1750:
1398:
1389:
1359:
1358:
1354:
3030:
Sevilla MA, Hermsen MA, Weiss MM, Grimbergen A, BalbĂn M, Llorente JL, Rodrigo JP, Suárez C (May 2009). "Chromosomal changes in sporadic and familial head and neck paragangliomas".
3182:"Variation at the NFATC2 locus increases the risk of thiazolidinedione-induced edema in the Diabetes REduction Assessment with ramipril and rosiglitazone Medication (DREAM) study"
3707:
2372:
Alston CL, Ceccatelli Berti C, Blakely EL, Oláhová M, He L, McMahon CJ, Olpin SE, Hargreaves IP, Nolli C, McFarland R, Goffrini P, O'Sullivan MJ, Taylor RW (August 2015).
264:
163:
3677:
3067:
Hendrickson SL, Lautenberger JA, Chinn LW, Malasky M, Sezgin E, Kingsley LA, Goedert JJ, Kirk GD, Gomperts ED, Buchbinder SP, Troyer JL, O'Brien SJ (September 2010).
4557:
3331:
3971:
3700:
3611:
4591:
4201:
3324:
2419:
706:
86:
687:
4734:
4045:
4040:
1099:
1758:
1362:
3582:
1773:
1377:
4392:
2857:"Relevance of germline mutation screening in both familial and sporadic head and neck paraganglioma for early diagnosis and clinical management"
2898:
Hensen EF, Jansen JC, Siemers MD, Oosterwijk JC, Vriends AH, Corssmit EP, Bayley JP, van der Mey AG, Cornelisse CJ, Devilee P (January 2010).
5153:
3262:
1933:
1915:
4604:
4600:
2900:"The Dutch founder mutation SDHD.D92Y shows a reduced penetrance for the development of paragangliomas in a large multigenerational family"
1789:
1776:
1762:
1393:
1380:
1366:
4819:
4550:
1792:
1396:
930:
250:
3464:
3383:
2441:
Bayley JP, Weiss MM, Grimbergen A, van
Brussel BT, Hes FJ, Jansen JC, Verhoef S, Devilee P, Corssmit EP, Vriends AH (September 2009).
2476:
Gaal J, Burnichon N, Korpershoek E, Roncelin I, Bertherat J, Plouin PF, de
Krijger RR, Gimenez-Roqueplo AP, Dinjens WN (March 2010).
4852:
4692:
4587:
937:
227:
1162:, loosely bound to the active site, free to exit the protein. The electrons derived from succinate tunnel along the relay in the
3897:
2374:"A recessive homozygous p.Asp92Gly SDHD mutation causes prenatal cardiomyopathy and a severe mitochondrial complex II deficiency"
1956:
1778:
1382:
1799:
1771:
1746:
1403:
1375:
1350:
5158:
2556:"Identification of a 4.9-kilo base-pair Alu-mediated founder SDHD deletion in two extended paraganglioma families from Austria"
2443:"Molecular characterization of novel germline deletions affecting SDHD and SDHC in pheochromocytoma and paraganglioma patients"
1902:
1881:
1784:
1747:
1388:
1351:
2774:
Eng C, Kiuru M, Fernandez MJ, Aaltonen LA (March 2003). "A role for mitochondrial enzymes in inherited neoplasia and beyond".
4652:
4609:
4543:
3759:
3621:
3451:
3439:
3388:
1291:
1241:
The exact mechanism for tumorigenesis is not determined, but it is suspected that malfunction of the SDH complex can cause a
149:
124:
5008:
2855:
Hermsen MA, Sevilla MA, Llorente JL, Weiss MM, Grimbergen A, Allonca E, Garcia-Inclán C, BalbĂn M, Suárez C (January 2010).
1898:
1287:
1265:
or a similar disorder called Cowden-like syndrome. These conditions are characterized by multiple tumor-like growths called
1294:(GIST). Those with Carney-Stratakis syndrome present with a noncancerous tumor associated with the nervous system called a
4687:
3460:
3180:
Bailey SD, Xie C, Do R, Montpetit A, Diaz R, Mohan V, Keavney B, Yusuf S, Gerstein HC, Engert JC, Anand S (October 2010).
1786:
1751:
1390:
1355:
5123:
1877:
4814:
4647:
3447:
1802:
1801:
1760:
1748:
1406:
1405:
1364:
1352:
1187:
radical species is formed. The second electron arrives from the cluster to provide full reduction of the ubiquinone to
1091:
1027:. Hereditary PGL-PCC syndrome is caused by a parental imprint of the SDHD gene. Screening can begin by 6 years of age.
66:
1770:
1374:
4677:
4011:
3291:: Crystal Structure of Mitochondrial Respiratory Complex II bound with 3-nitropropionate and 2-thenoyltrifluoroacetone
1800:
1753:
1404:
1357:
1155:
1787:
1391:
2159:
Horsefield R, Yankovskaya V, Sexton G, Whittingham W, Shiomi K, Omura S, Byrne B, Cecchini G, Iwata S (March 2006).
1805:
1788:
1782:
1777:
1775:
1767:
1409:
1392:
1386:
1381:
1379:
1371:
263:
162:
4809:
4697:
4657:
4025:
3933:
3849:
3820:
1806:
1803:
1766:
1410:
1407:
1370:
4993:
4570:
1797:
1401:
1319:
5109:
5096:
5083:
5070:
5057:
5044:
5031:
4765:
4751:
4707:
4634:
4619:
4583:
4421:
4259:
4059:
3548:
3255:
2668:"Tumor risks and genotype-phenotype-proteotype analysis in 358 patients with germline mutations in SDHB and SDHD"
256:
155:
5003:
1808:
1807:
1798:
1768:
1763:
1412:
1411:
1402:
1372:
1367:
4957:
4900:
4574:
3878:
3764:
3543:
3503:
3378:
1804:
1790:
1772:
1765:
1756:
1752:
1749:
1408:
1394:
1376:
1369:
1360:
1356:
1353:
1024:
751:
74:
2513:"Development and validation of a comprehensive mutation and deletion detection assay for SDHB, SDHC, and SDHD"
1795:
1779:
1774:
1769:
1764:
1399:
1383:
1378:
1373:
1368:
1167:
2812:
Smith GD, Lawlor DA, Munroe PB, Day IN, Kivimaki M, Whittaker J, Humphries SE, Hingorani AD (November 2009).
2247:
1783:
1780:
1759:
1387:
1384:
1363:
4905:
4719:
4104:
3782:
3568:
3484:
3395:
1083:
732:
4770:
4481:
4290:
3527:
3476:
3443:
1827:
3235:
4926:
4845:
4672:
3980:
3791:
3735:
3723:
3421:
1218:
were first linked to hereditary paraganglioma in 2000. Since then, it has been shown that mutations in
138:
4998:
2814:"Gene-centric association signals for lipids and apolipoproteins identified via the HumanCVD BeadChip"
4682:
4614:
4406:
3592:
3522:
3410:
3248:
3080:
2210:
53:
4962:
3864:
3672:
888:
884:
880:
829:
825:
821:
57:, CBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, succinate dehydrogenase complex subunit D, MC2DN3
4535:
4895:
4760:
3919:
3563:
3146:
3055:
3018:
2980:
2799:
2697:
2653:
2615:
2354:
2141:
98:
913:
892:
854:
833:
3306:: Crystal Structure of Mitochondrial Respiratory Complex II from porcine heart at 2.4 Angstroms
2093:"SDHD - Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial"
5163:
4787:
4662:
3351:
3211:
3168:
3138:
3108:
3047:
3010:
2972:
2929:
2886:
2843:
2791:
2762:
2727:
2689:
2645:
2607:
2577:
2542:
2499:
2464:
2403:
2346:
2226:
2182:
2133:
2074:
2024:
1988:
1242:
1211:
1082:
The SDHD protein is one of the two integral transmembrane subunits anchoring the four-subunit
1020:
46:
2554:
Janecke AR, Willett-Brozick JE, Karas C, Hasipek M, Loeffler-Ragg J, Baysal BE (March 2010).
4941:
4936:
4910:
4838:
4667:
4356:
4133:
4119:
3646:
3641:
3514:
3368:
3201:
3193:
3130:
3121:"Clinical predictors and algorithm for the genetic diagnosis of pheochromocytoma patients".
3098:
3088:
3039:
3002:
2962:
2954:
2941:
Brière JJ, Favier J, El
Ghouzzi V, Djouadi F, BĂ©nit P, Gimenez AP, Rustin P (October 2005).
2919:
2911:
2876:
2868:
2833:
2825:
2783:
2752:
2719:
2679:
2637:
2599:
2567:
2532:
2524:
2489:
2454:
2393:
2385:
2338:
2218:
2172:
2123:
2064:
2056:
2016:
1980:
1303:
1299:
1227:
1044:
343:
274:
218:
173:
4988:
4972:
4885:
4519:
2511:
Milosevic D, Lundquist P, Cradic K, Vidal-Folch N, Huynh T, Pacak K, Grebe SK (May 2010).
2302:
1262:
1087:
318:
94:
3316:
3084:
2967:
2942:
2214:
601:
mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)
5137:
5026:
4967:
4566:
4322:
3692:
3206:
3181:
3103:
3068:
2924:
2899:
2881:
2856:
2838:
2813:
2537:
2512:
2398:
2373:
2069:
2044:
1315:
1275:
2528:
621:
616:
600:
595:
590:
585:
580:
575:
559:
554:
549:
544:
539:
534:
5147:
4931:
4890:
4472:
4174:
4148:
3744:
2757:
2740:
2478:"Isocitrate dehydrogenase mutations are rare in pheochromocytomas and paragangliomas"
2423:
2092:
2045:"Integration of cardiac proteome biology and medicine by a specialized knowledgebase"
1848:
1831:
1818:
1295:
1271:
1238:
mutation related tumors are more typically benign, originating in the head and neck.
1207:
1179:
1151:
1127:
1107:
1095:
1072:
1040:
521:
3231:
GeneReviews/NCBI/NIH/UW entry on
Hereditary Paraganglioma-Pheochromocytoma Syndromes
3069:"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression"
2984:
2803:
2701:
2619:
2358:
2145:
1138:
to the inner mitochondrial membrane. The SDHC/SDHD dimer provides binding sites for
78:
4880:
4235:
3995:
3587:
3573:
3495:
3150:
3059:
3022:
2657:
1068:
1064:
1060:
1056:
336:
115:
3134:
2723:
2342:
2060:
3093:
3043:
2222:
102:
5104:
5039:
4875:
4090:
3806:
1938:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1920:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1250:
1184:
1131:
1103:
1076:
1052:
5132:
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2128:
2111:
419:
4371:
4318:
4300:
4244:
3431:
3347:
3230:
3006:
2958:
2641:
2389:
1331:
Click on genes, proteins and metabolites below to link to respective articles.
1266:
1171:
1139:
1135:
1048:
235:
132:
82:
1306:, is acquired during a person's lifetime and is present only in tumor cells.
5078:
5052:
3373:
2112:"Crystal structure of mitochondrial respiratory membrane protein complex II"
1261:
At least five variants in the SDHD gene have been identified in people with
1188:
1147:
651:
479:
357:
302:
289:
201:
188:
90:
3215:
3172:
3142:
3112:
3051:
3014:
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2933:
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2731:
2693:
2649:
2611:
2603:
2581:
2546:
2503:
2468:
2407:
2350:
2230:
2186:
2177:
2160:
2137:
2078:
2915:
2872:
2028:
1992:
1742:
1346:
977:
972:
4524:
4269:
4264:
3902:
3727:
3405:
2572:
2555:
2494:
2477:
1175:
1159:
961:
796:
777:
2459:
2442:
2110:
Sun F, Huo X, Zhai Y, Wang A, Xu J, Su D, Bartlam M, Rao Z (July 2005).
1984:
1234:
mutations often lead to metastatic disease that is extra-adrenal, while
1230:. Notably, the tumor spectrum is different for the different mutations.
4711:
2684:
2667:
997:
763:
718:
3197:
2020:
5091:
4861:
4802:
4797:
4792:
4775:
4638:
4304:
4217:
3354:
1290:, a hereditary form of a cancer of the gastrointestinal tract called
1254:
1245:
response in the cell that leads to tumor formation. Mutations in the
1016:
945:
673:
2787:
2276:
32:
5065:
4780:
4514:
4431:
4426:
4342:
4337:
4158:
3953:
3948:
3914:
3907:
3835:
3656:
3651:
990:
Succinate dehydrogenase cytochrome b small subunit, mitochondrial
1178:
oxygen of ubiquinone is oriented at the active site (image 4) by
636:
632:
4729:
4724:
4489:
4454:
4376:
4153:
3893:
3636:
3631:
3626:
3616:
3606:
3399:
1246:
1203:
1163:
1143:
1115:
1111:
1036:
1004:
70:
4834:
4539:
3696:
3320:
3244:
1114:
electron transport subunit which, in turn, is connected to the
4438:
4295:
4285:
4074:
3240:
1142:
and water during electron transport at
Complex II. Initially,
1741:
1345:
3159:
apparently sporadic pheochromocytomas and paragangliomas".
1846:
The interactive pathway map can be edited at WikiPathways:
1174:
molecule at the active site in the SDHC/SDHD dimer. The O1
1075:. The SDHD gene produces a 17 kDa protein composed of 159
4830:
622:
mitochondrial electron transport, succinate to ubiquinone
326:
2097:
Cardiac
Organellar Protein Atlas Knowledgebase (COPaKB)
1286:
gene have been found in a small number of people with
5121:
491:
4625:
Dihydroxymethyloxo-tetrahydroquinoline dehydrogenase
2482:
The
Journal of Clinical Endocrinology and Metabolism
1170:. The electrons are then transferred to an awaiting
5017:
4981:
4950:
4919:
4868:
4750:
4706:
4633:
4582:
4507:
4480:
4471:
4447:
4414:
4405:
4385:
4364:
4355:
4330:
4317:
4278:
4252:
4243:
4234:
4210:
4194:
4187:
4167:
4141:
4132:
4112:
4103:
4083:
4067:
4058:
4033:
4024:
4004:
3988:
3979:
3970:
3941:
3932:
3886:
3877:
3857:
3848:
3828:
3819:
3799:
3790:
3781:
3752:
3743:
3734:
3665:
3556:
3540:
3512:
3493:
3474:
3429:
3420:
3361:
906:
873:
847:
814:
1894:
1892:
1890:
1873:
1871:
1869:
273:
172:
3678:Electron-transferring-flavoprotein dehydrogenase
2739:Martin TP, Irving RM, Maher ER (February 2007).
3583:Complex III/Coenzyme Q - cytochrome c reductase
1745:
1349:
18:Protein-coding gene in the species Homo sapiens
3236:SDHD from the HUGO Gene Nomenclature Committee
1957:"Entrez Gene: succinate dehydrogenase complex"
1899:GRCm38: Ensembl release 89: ENSMUSG00000000171
4846:
4551:
3708:
3332:
3256:
2943:"Succinate dehydrogenase deficiency in human"
550:succinate dehydrogenase (ubiquinone) activity
8:
2271:
2269:
2248:"Hereditary paraganglioma-pheochromocytoma"
1878:GRCh38: Ensembl release 89: ENSG00000204370
4853:
4839:
4831:
4558:
4544:
4536:
4477:
4411:
4361:
4327:
4249:
4240:
4191:
4138:
4109:
4064:
4030:
3985:
3976:
3938:
3883:
3854:
3825:
3796:
3787:
3749:
3740:
3715:
3701:
3693:
3553:
3426:
3339:
3325:
3317:
3263:
3249:
3241:
2741:"The genetics of paragangliomas: a review"
2420:United States National Library of Medicine
2242:
2240:
1226:can cause paranglioma as well as familial
1015:. Succinate dehydrogenase is an important
647:
517:
404:Skeletal muscle tissue of rectus abdominis
314:
213:
110:
3205:
3102:
3092:
2966:
2923:
2880:
2837:
2756:
2683:
2571:
2536:
2493:
2458:
2397:
2176:
2127:
2068:
994:succinate dehydrogenase complex subunit D
2418:This article incorporates text from the
1951:
1949:
1947:
400:Skeletal muscle tissue of biceps brachii
5128:
3278:
1865:
1839:
458:cardiac muscle tissue of left ventricle
1007:. Names previously used for SDHD were
22:
1126:SDHD forms part of the transmembrane
278:
239:
234:
177:
136:
131:
7:
4601:Enoyl-acyl carrier protein reductase
3032:Otolaryngology–Head and Neck Surgery
2947:Cellular and Molecular Life Sciences
4820:3-oxo-5beta-steroid 4-dehydrogenase
4815:Isovaleryl coenzyme A dehydrogenase
2283:. U.S. National Library of Medicine
2254:. U.S. National Library of Medicine
2165:The Journal of Biological Chemistry
3569:Complex II/Succinate dehydrogenase
3465:Pyruvate dehydrogenase phosphatase
2904:European Journal of Human Genetics
2818:American Journal of Human Genetics
903:
870:
844:
811:
787:
768:
742:
723:
697:
678:
496:
414:
352:
331:
14:
4693:Pyrroloquinoline-quinone synthase
2590:pheochromocytoma/paraganglioma".
2529:10.1016/j.clinbiochem.2010.01.016
1000:that in humans is encoded by the
5131:
3296:
3281:
2758:10.1111/j.1365-2273.2007.01378.x
540:succinate dehydrogenase activity
262:
255:
249:
226:
161:
154:
148:
123:
31:
3593:Complex IV/Cytochrome c oxidase
2309:. GeneCards Human Gene Database
4653:Coproporphyrinogen III oxidase
4610:7-Dehydrocholesterol reductase
3440:Pyruvate dehydrogenase complex
2592:Hormone and Metabolic Research
2009:Cytogenetics and Cell Genetics
1292:gastrointestinal stromal tumor
1166:subunit until they reach the
576:integral component of membrane
480:More reference expression data
1:
4688:Tryptophan alpha,beta-oxidase
3461:Pyruvate dehydrogenase kinase
3135:10.1158/1078-0432.CCR-09-1237
2724:10.1016/j.humpath.2009.12.005
2343:10.1136/jmedgenet-2013-101932
2061:10.1161/CIRCRESAHA.113.301151
247:
146:
5154:Genes on human chromosome 11
4648:Dihydroorotate dehydrogenase
3972:Intracellular signaling P+Ps
3564:Complex I/NADH dehydrogenase
3094:10.1371/journal.pone.0012862
3044:10.1016/j.otohns.2009.01.004
2223:10.1126/science.287.5454.848
1092:mitochondrial inner membrane
581:mitochondrial inner membrane
4678:Tetrahydroberberine oxidase
2995:Clinical Autonomic Research
2331:Journal of Medical Genetics
1183:electron reduction step, a
5180:
4810:Glutaryl-CoA dehydrogenase
4698:L-galactonolactone oxidase
4658:Protoporphyrinogen oxidase
4026:TGF beta signaling pathway
3934:JAK-STAT signaling pathway
3850:TGF beta signaling pathway
3821:Hedgehog signaling pathway
3384:Oxoglutarate dehydrogenase
2830:10.1016/j.ajhg.2009.10.014
2129:10.1016/j.cell.2005.05.025
1090:to the matrix side of the
931:Chr 11: 112.09 – 112.12 Mb
535:electron transfer activity
466:sternocleidomastoid muscle
396:mucosa of transverse colon
5009:Michaelis–Menten kinetics
4766:Butyryl-CoA dehydrogenase
4620:2,4 Dienoyl-CoA reductase
4060:Akt/PKB signaling pathway
3549:oxidative phosphorylation
3276:
3007:10.1007/s10286-009-0028-z
2959:10.1007/s00018-005-5237-6
2642:10.1007/s12020-009-9178-y
2560:Journal of Human Genetics
2390:10.1007/s00439-015-1568-z
1934:"Mouse PubMed Reference:"
1916:"Human PubMed Reference:"
1288:Carney–Stratakis syndrome
1047:11q23 and it spans 8,978
976:
971:
967:
960:
944:
925:
910:
877:
866:
851:
818:
807:
794:
790:
775:
771:
762:
749:
745:
730:
726:
717:
704:
700:
685:
681:
672:
657:
650:
646:
630:
520:
516:
504:
499:
490:
477:
426:
417:
364:
355:
325:
317:
313:
296:
283:
246:
225:
216:
212:
195:
182:
145:
122:
113:
109:
64:
61:
51:
44:
39:
30:
25:
4901:Diffusion-limited enzyme
3879:Receptor tyrosine kinase
3544:electron transport chain
3504:Methylmalonyl-CoA mutase
3379:Isocitrate dehydrogenase
3123:Clinical Cancer Research
2447:Endocrine-Related Cancer
1973:Human Molecular Genetics
1025:electron transport chain
617:tricarboxylic acid cycle
4720:Succinate dehydrogenase
4105:Hippo signaling pathway
3485:Glutamate dehydrogenase
3396:Succinate dehydrogenase
3389:Succinyl CoA synthetase
2745:Clinical Otolaryngology
2281:Genetics Home Reference
2252:Genetics Home Reference
1326:Interactive pathway map
1222:and to a lesser degree
1084:succinate dehydrogenase
938:Chr 9: 50.51 – 50.52 Mb
442:myocardium of ventricle
434:interventricular septum
392:mucosa of sigmoid colon
5159:Tumor suppressor genes
4771:Acyl CoA dehydrogenase
4120:Neurofibromin 2/Merlin
3724:Tumor suppressor genes
3528:Aspartate transaminase
2776:Nature Reviews. Cancer
2604:10.1055/s-0029-1202814
2178:10.1074/jbc.m508173200
1811:
1415:
1158:binding site, leaving
992:(CybS), also known as
586:mitochondrial envelope
4994:Eadie–Hofstee diagram
4927:Allosteric regulation
4673:Dihydrouracil oxidase
4571:CH–CH oxidoreductases
3981:Wnt signaling pathway
3792:Wnt signaling pathway
3161:Endokrynologia Polska
2916:10.1038/ejhg.2009.112
2873:10.3233/CLO-2009-0498
2517:Clinical Biochemistry
1810:
1414:
1320:Kearns–Sayre syndrome
1195:Clinical significance
139:Chromosome 11 (human)
5004:Lineweaver–Burk plot
4683:Secologanin synthase
4615:Biliverdin reductase
4407:Transcription factor
3523:Pyruvate carboxylase
3411:Malate dehydrogenase
2573:10.1038/jhg.2009.142
2495:10.1210/jc.2009-2170
2049:Circulation Research
1817:|alt=TCACycle_WP78
1110:is connected to the
241:Chromosome 9 (mouse)
4605:Enoyl ACP reductase
4482:Apoptosis inhibitor
3865:TGF beta receptor 2
3673:Alternative oxidase
3477:α-ketoglutaric acid
3085:2010PLoSO...512862H
2666:ER (January 2010).
2460:10.1677/ERC-09-0084
2215:2000Sci...287..848B
1206:can cause familial
1168:iron–sulfur cluster
4963:Enzyme superfamily
4896:Enzyme promiscuity
4761:Fumarate reductase
2953:(19–20): 2317–24.
2685:10.1002/humu.21136
2422:, which is in the
1812:
1416:
1212:Germline mutations
752:ENSMUSG00000000171
610:Biological process
569:Cellular component
560:ubiquinone binding
528:Molecular function
462:thoracic diaphragm
446:intercostal muscle
438:extraocular muscle
5119:
5118:
4828:
4827:
4754:: Other acceptors
4663:Bilirubin oxidase
4533:
4532:
4503:
4502:
4467:
4466:
4463:
4462:
4401:
4400:
4351:
4350:
4313:
4312:
4230:
4229:
4226:
4225:
4183:
4182:
4128:
4127:
4099:
4098:
4054:
4053:
4020:
4019:
3966:
3965:
3962:
3961:
3928:
3927:
3873:
3872:
3844:
3843:
3815:
3814:
3777:
3776:
3773:
3772:
3690:
3689:
3686:
3685:
3536:
3535:
3352:Citric acid cycle
3314:
3313:
3198:10.2337/dc10-0452
2861:Cellular Oncology
2307:www.genecards.org
2021:10.1159/000134700
1985:10.1093/hmg/1.1.7
1282:Mutations in the
1199:Mutations in the
1055:for this gene on
1021:citric acid cycle
987:
986:
983:
982:
956:
955:
921:
920:
900:
899:
862:
861:
841:
840:
803:
802:
784:
783:
758:
757:
739:
738:
713:
712:
694:
693:
642:
641:
545:metal ion binding
512:
511:
508:
507:
486:
485:
473:
472:
411:
410:
309:
308:
208:
207:
5171:
5136:
5135:
5127:
4999:Hanes–Woolf plot
4942:Enzyme activator
4937:Enzyme inhibitor
4911:Enzyme catalysis
4855:
4848:
4841:
4832:
4668:Acyl-CoA oxidase
4560:
4553:
4546:
4537:
4478:
4412:
4362:
4357:Ubiquitin ligase
4328:
4250:
4241:
4192:
4139:
4134:MAPK/ERK pathway
4110:
4065:
4031:
3986:
3977:
3939:
3884:
3855:
3826:
3797:
3788:
3750:
3741:
3717:
3710:
3703:
3694:
3554:
3515:oxaloacetic acid
3427:
3369:Citrate synthase
3341:
3334:
3327:
3318:
3300:
3285:
3265:
3258:
3251:
3242:
3219:
3209:
3176:
3154:
3116:
3106:
3096:
3063:
3026:
2988:
2970:
2937:
2927:
2894:
2884:
2851:
2841:
2807:
2770:
2760:
2735:
2705:
2687:
2661:
2623:
2585:
2575:
2550:
2540:
2507:
2497:
2472:
2462:
2412:
2411:
2401:
2369:
2363:
2362:
2325:
2319:
2318:
2316:
2314:
2299:
2293:
2292:
2290:
2288:
2273:
2264:
2263:
2261:
2259:
2244:
2235:
2234:
2209:(5454): 848–51.
2197:
2191:
2190:
2180:
2156:
2150:
2149:
2131:
2107:
2101:
2100:
2089:
2083:
2082:
2072:
2039:
2033:
2032:
2003:
1997:
1996:
1967:
1961:
1960:
1953:
1942:
1941:
1930:
1924:
1923:
1912:
1906:
1896:
1885:
1875:
1853:
1852:
1844:
1737:
1732:
1727:
1722:
1717:
1712:
1707:
1702:
1697:
1692:
1687:
1682:
1677:
1672:
1667:
1662:
1657:
1652:
1647:
1642:
1637:
1632:
1627:
1622:
1617:
1612:
1607:
1602:
1597:
1592:
1587:
1582:
1577:
1572:
1567:
1562:
1557:
1552:
1547:
1542:
1537:
1532:
1527:
1522:
1517:
1512:
1507:
1502:
1497:
1492:
1487:
1482:
1477:
1472:
1467:
1462:
1457:
1452:
1447:
1442:
1437:
1432:
1427:
1422:
1304:somatic mutation
1300:pheochromocytoma
1228:pheochromocytoma
969:
968:
940:
933:
916:
904:
895:
871:
867:RefSeq (protein)
857:
845:
836:
812:
788:
769:
743:
724:
698:
679:
648:
518:
497:
482:
454:digastric muscle
422:
420:Top expressed in
415:
360:
358:Top expressed in
353:
332:
315:
305:
292:
281:
266:
259:
253:
242:
230:
214:
204:
191:
180:
165:
158:
152:
141:
127:
111:
105:
103:SDHD - orthologs
56:
49:
35:
23:
5179:
5178:
5174:
5173:
5172:
5170:
5169:
5168:
5144:
5143:
5142:
5130:
5122:
5120:
5115:
5027:Oxidoreductases
5013:
4989:Enzyme kinetics
4977:
4973:List of enzymes
4946:
4915:
4886:Catalytic triad
4864:
4859:
4829:
4824:
4746:
4702:
4629:
4578:
4567:Oxidoreductases
4564:
4534:
4529:
4508:Other/ungrouped
4499:
4459:
4443:
4397:
4381:
4347:
4309:
4274:
4222:
4206:
4179:
4175:Neurofibromin 1
4163:
4124:
4095:
4079:
4050:
4016:
4000:
3958:
3924:
3869:
3840:
3811:
3769:
3730:
3721:
3691:
3682:
3661:
3603:
3577:
3547:
3542:
3532:
3508:
3489:
3470:
3416:
3357:
3345:
3315:
3310:
3307:
3301:
3292:
3286:
3272:
3269:
3227:
3222:
3179:
3157:
3129:(20): 6378–85.
3119:
3066:
3029:
2991:
2940:
2897:
2854:
2810:
2788:10.1038/nrc1013
2773:
2738:
2712:Human Pathology
2708:
2664:
2626:
2588:
2553:
2510:
2475:
2440:
2436:
2434:Further reading
2430:
2415:
2371:
2370:
2366:
2327:
2326:
2322:
2312:
2310:
2301:
2300:
2296:
2286:
2284:
2275:
2274:
2267:
2257:
2255:
2246:
2245:
2238:
2199:
2198:
2194:
2171:(11): 7309–16.
2158:
2157:
2153:
2109:
2108:
2104:
2091:
2090:
2086:
2041:
2040:
2036:
2005:
2004:
2000:
1969:
1968:
1964:
1955:
1954:
1945:
1932:
1931:
1927:
1914:
1913:
1909:
1897:
1888:
1876:
1867:
1863:
1858:
1857:
1856:
1849:"TCACycle_WP78"
1847:
1845:
1841:
1836:
1835:
1834:
1830:TCACycle_WP78
1829:
1824:
1823:
1822:
1816:
1815:
1814:
1813:
1809:
1739:
1738:
1735:
1733:
1730:
1728:
1725:
1723:
1720:
1718:
1715:
1713:
1710:
1708:
1705:
1703:
1700:
1698:
1695:
1693:
1690:
1688:
1685:
1683:
1680:
1678:
1675:
1673:
1670:
1668:
1665:
1663:
1660:
1658:
1655:
1653:
1650:
1648:
1645:
1643:
1640:
1638:
1635:
1633:
1630:
1628:
1625:
1623:
1620:
1618:
1615:
1613:
1610:
1608:
1605:
1603:
1600:
1598:
1595:
1593:
1590:
1588:
1585:
1583:
1580:
1578:
1575:
1573:
1570:
1568:
1565:
1563:
1560:
1558:
1555:
1553:
1550:
1548:
1545:
1543:
1540:
1538:
1535:
1533:
1530:
1528:
1525:
1523:
1520:
1518:
1515:
1513:
1510:
1508:
1505:
1503:
1500:
1498:
1495:
1493:
1490:
1488:
1485:
1483:
1480:
1478:
1475:
1473:
1470:
1468:
1465:
1463:
1460:
1458:
1455:
1453:
1450:
1448:
1445:
1443:
1440:
1438:
1435:
1433:
1430:
1428:
1425:
1423:
1420:
1417:
1413:
1328:
1263:Cowden syndrome
1197:
1124:
1088:protein complex
1039:is located on
1033:
978:View/Edit Mouse
973:View/Edit Human
936:
929:
926:Location (UCSC)
912:
891:
887:
883:
879:
853:
832:
828:
824:
820:
733:ENSG00000204370
626:
605:
564:
478:
469:
464:
460:
456:
452:
450:cardiac muscles
448:
444:
440:
436:
432:
418:
407:
402:
398:
394:
390:
388:right ventricle
386:
384:mucosa of colon
382:
378:
374:
370:
356:
300:
287:
279:
269:
268:
267:
260:
240:
217:Gene location (
199:
186:
178:
168:
167:
166:
159:
137:
114:Gene location (
65:
52:
45:
19:
12:
11:
5:
5177:
5175:
5167:
5166:
5161:
5156:
5146:
5145:
5141:
5140:
5117:
5116:
5114:
5113:
5100:
5087:
5074:
5061:
5048:
5035:
5021:
5019:
5015:
5014:
5012:
5011:
5006:
5001:
4996:
4991:
4985:
4983:
4979:
4978:
4976:
4975:
4970:
4965:
4960:
4954:
4952:
4951:Classification
4948:
4947:
4945:
4944:
4939:
4934:
4929:
4923:
4921:
4917:
4916:
4914:
4913:
4908:
4903:
4898:
4893:
4888:
4883:
4878:
4872:
4870:
4866:
4865:
4860:
4858:
4857:
4850:
4843:
4835:
4826:
4825:
4823:
4822:
4817:
4812:
4807:
4806:
4805:
4800:
4795:
4785:
4784:
4783:
4778:
4768:
4763:
4757:
4755:
4748:
4747:
4745:
4744:
4743:
4742:
4737:
4732:
4727:
4716:
4714:
4704:
4703:
4701:
4700:
4695:
4690:
4685:
4680:
4675:
4670:
4665:
4660:
4655:
4650:
4644:
4642:
4631:
4630:
4628:
4627:
4622:
4617:
4612:
4607:
4597:
4595:
4580:
4579:
4565:
4563:
4562:
4555:
4548:
4540:
4531:
4530:
4528:
4527:
4522:
4517:
4511:
4509:
4505:
4504:
4501:
4500:
4498:
4497:
4492:
4486:
4484:
4475:
4469:
4468:
4465:
4464:
4461:
4460:
4458:
4457:
4451:
4449:
4445:
4444:
4442:
4441:
4436:
4435:
4434:
4429:
4418:
4416:
4409:
4403:
4402:
4399:
4398:
4396:
4395:
4389:
4387:
4383:
4382:
4380:
4379:
4374:
4368:
4366:
4359:
4353:
4352:
4349:
4348:
4346:
4345:
4340:
4334:
4332:
4325:
4315:
4314:
4311:
4310:
4308:
4307:
4298:
4293:
4288:
4282:
4280:
4276:
4275:
4273:
4272:
4267:
4262:
4256:
4254:
4247:
4238:
4232:
4231:
4228:
4227:
4224:
4223:
4221:
4220:
4214:
4212:
4208:
4207:
4205:
4204:
4198:
4196:
4189:
4185:
4184:
4181:
4180:
4178:
4177:
4171:
4169:
4165:
4164:
4162:
4161:
4156:
4151:
4145:
4143:
4136:
4130:
4129:
4126:
4125:
4123:
4122:
4116:
4114:
4107:
4101:
4100:
4097:
4096:
4094:
4093:
4087:
4085:
4081:
4080:
4078:
4077:
4071:
4069:
4062:
4056:
4055:
4052:
4051:
4049:
4048:
4043:
4037:
4035:
4028:
4022:
4021:
4018:
4017:
4015:
4014:
4008:
4006:
4002:
4001:
3999:
3998:
3992:
3990:
3983:
3974:
3968:
3967:
3964:
3963:
3960:
3959:
3957:
3956:
3951:
3945:
3943:
3936:
3930:
3929:
3926:
3925:
3923:
3922:
3917:
3912:
3911:
3910:
3905:
3890:
3888:
3881:
3875:
3874:
3871:
3870:
3868:
3867:
3861:
3859:
3852:
3846:
3845:
3842:
3841:
3839:
3838:
3832:
3830:
3823:
3817:
3816:
3813:
3812:
3810:
3809:
3803:
3801:
3794:
3785:
3779:
3778:
3775:
3774:
3771:
3770:
3768:
3767:
3762:
3756:
3754:
3747:
3745:Growth factors
3738:
3732:
3731:
3722:
3720:
3719:
3712:
3705:
3697:
3688:
3687:
3684:
3683:
3681:
3680:
3675:
3669:
3667:
3663:
3662:
3660:
3659:
3654:
3649:
3644:
3639:
3634:
3629:
3624:
3619:
3614:
3609:
3601:
3596:
3595:
3590:
3585:
3580:
3575:
3571:
3566:
3560:
3558:
3551:
3538:
3537:
3534:
3533:
3531:
3530:
3525:
3519:
3517:
3510:
3509:
3507:
3506:
3500:
3498:
3491:
3490:
3488:
3487:
3481:
3479:
3472:
3471:
3469:
3468:
3459:(regulated by
3456:
3455:
3436:
3434:
3424:
3418:
3417:
3415:
3414:
3408:
3403:
3392:
3391:
3386:
3381:
3376:
3371:
3365:
3363:
3359:
3358:
3346:
3344:
3343:
3336:
3329:
3321:
3312:
3311:
3309:
3308:
3302:
3295:
3293:
3287:
3280:
3277:
3274:
3273:
3270:
3268:
3267:
3260:
3253:
3245:
3239:
3238:
3233:
3226:
3225:External links
3223:
3221:
3220:
3192:(10): 2250–3.
3177:
3155:
3117:
3064:
3027:
2989:
2938:
2895:
2852:
2808:
2782:(3): 193–202.
2771:
2736:
2706:
2672:Human Mutation
2662:
2624:
2586:
2551:
2523:(7–8): 700–4.
2508:
2473:
2437:
2435:
2432:
2414:
2413:
2378:Human Genetics
2364:
2320:
2294:
2265:
2236:
2192:
2151:
2122:(7): 1043–57.
2102:
2084:
2055:(9): 1043–53.
2034:
2015:(1–2): 132–8.
1998:
1962:
1943:
1925:
1907:
1886:
1864:
1862:
1859:
1855:
1854:
1838:
1837:
1826:
1825:
1740:
1734:
1729:
1724:
1719:
1714:
1709:
1704:
1699:
1694:
1689:
1684:
1679:
1674:
1669:
1664:
1659:
1654:
1649:
1644:
1639:
1634:
1629:
1624:
1619:
1614:
1609:
1604:
1599:
1594:
1589:
1584:
1579:
1574:
1569:
1564:
1559:
1554:
1549:
1544:
1539:
1534:
1529:
1524:
1519:
1514:
1509:
1504:
1499:
1494:
1489:
1484:
1479:
1474:
1469:
1464:
1459:
1454:
1449:
1444:
1439:
1434:
1429:
1424:
1419:
1418:
1344:
1343:
1342:
1341:
1339:
1338:
1337:
1336:
1335:
1334:
1327:
1324:
1316:Leigh syndrome
1196:
1193:
1123:
1120:
1032:
1029:
985:
984:
981:
980:
975:
965:
964:
958:
957:
954:
953:
951:
949:
942:
941:
934:
927:
923:
922:
919:
918:
908:
907:
901:
898:
897:
875:
874:
868:
864:
863:
860:
859:
849:
848:
842:
839:
838:
816:
815:
809:
805:
804:
801:
800:
792:
791:
785:
782:
781:
773:
772:
766:
760:
759:
756:
755:
747:
746:
740:
737:
736:
728:
727:
721:
715:
714:
711:
710:
702:
701:
695:
692:
691:
683:
682:
676:
670:
669:
664:
659:
655:
654:
644:
643:
640:
639:
628:
627:
625:
624:
619:
613:
611:
607:
606:
604:
603:
598:
593:
588:
583:
578:
572:
570:
566:
565:
563:
562:
557:
552:
547:
542:
537:
531:
529:
525:
524:
514:
513:
510:
509:
506:
505:
502:
501:
494:
488:
487:
484:
483:
475:
474:
471:
470:
468:
467:
463:
459:
455:
451:
447:
443:
439:
435:
431:
427:
424:
423:
412:
409:
408:
406:
405:
401:
397:
393:
389:
385:
381:
380:biceps brachii
377:
373:
369:
368:jejunal mucosa
365:
362:
361:
349:
348:
340:
329:
323:
322:
319:RNA expression
311:
310:
307:
306:
298:
294:
293:
285:
282:
277:
271:
270:
261:
254:
248:
244:
243:
238:
232:
231:
223:
222:
210:
209:
206:
205:
197:
193:
192:
184:
181:
176:
170:
169:
160:
153:
147:
143:
142:
135:
129:
128:
120:
119:
107:
106:
63:
59:
58:
50:
42:
41:
37:
36:
28:
27:
17:
13:
10:
9:
6:
4:
3:
2:
5176:
5165:
5162:
5160:
5157:
5155:
5152:
5151:
5149:
5139:
5134:
5129:
5125:
5111:
5107:
5106:
5101:
5098:
5094:
5093:
5088:
5085:
5081:
5080:
5075:
5072:
5068:
5067:
5062:
5059:
5055:
5054:
5049:
5046:
5042:
5041:
5036:
5033:
5029:
5028:
5023:
5022:
5020:
5016:
5010:
5007:
5005:
5002:
5000:
4997:
4995:
4992:
4990:
4987:
4986:
4984:
4980:
4974:
4971:
4969:
4968:Enzyme family
4966:
4964:
4961:
4959:
4956:
4955:
4953:
4949:
4943:
4940:
4938:
4935:
4933:
4932:Cooperativity
4930:
4928:
4925:
4924:
4922:
4918:
4912:
4909:
4907:
4904:
4902:
4899:
4897:
4894:
4892:
4891:Oxyanion hole
4889:
4887:
4884:
4882:
4879:
4877:
4874:
4873:
4871:
4867:
4863:
4856:
4851:
4849:
4844:
4842:
4837:
4836:
4833:
4821:
4818:
4816:
4813:
4811:
4808:
4804:
4801:
4799:
4796:
4794:
4791:
4790:
4789:
4786:
4782:
4779:
4777:
4774:
4773:
4772:
4769:
4767:
4764:
4762:
4759:
4758:
4756:
4753:
4749:
4741:
4738:
4736:
4733:
4731:
4728:
4726:
4723:
4722:
4721:
4718:
4717:
4715:
4713:
4709:
4705:
4699:
4696:
4694:
4691:
4689:
4686:
4684:
4681:
4679:
4676:
4674:
4671:
4669:
4666:
4664:
4661:
4659:
4656:
4654:
4651:
4649:
4646:
4645:
4643:
4640:
4636:
4632:
4626:
4623:
4621:
4618:
4616:
4613:
4611:
4608:
4606:
4602:
4599:
4598:
4596:
4593:
4589:
4585:
4581:
4576:
4572:
4568:
4561:
4556:
4554:
4549:
4547:
4542:
4541:
4538:
4526:
4523:
4521:
4518:
4516:
4513:
4512:
4510:
4506:
4496:
4493:
4491:
4488:
4487:
4485:
4483:
4479:
4476:
4474:
4473:Mitochondrion
4470:
4456:
4453:
4452:
4450:
4446:
4440:
4437:
4433:
4430:
4428:
4425:
4424:
4423:
4420:
4419:
4417:
4413:
4410:
4408:
4404:
4394:
4391:
4390:
4388:
4384:
4378:
4375:
4373:
4370:
4369:
4367:
4363:
4360:
4358:
4354:
4344:
4341:
4339:
4336:
4335:
4333:
4329:
4326:
4324:
4320:
4316:
4306:
4302:
4299:
4297:
4294:
4292:
4289:
4287:
4284:
4283:
4281:
4277:
4271:
4268:
4266:
4263:
4261:
4258:
4257:
4255:
4251:
4248:
4246:
4242:
4239:
4237:
4233:
4219:
4216:
4215:
4213:
4209:
4203:
4200:
4199:
4197:
4193:
4190:
4188:Other/unknown
4186:
4176:
4173:
4172:
4170:
4166:
4160:
4157:
4155:
4152:
4150:
4147:
4146:
4144:
4140:
4137:
4135:
4131:
4121:
4118:
4117:
4115:
4111:
4108:
4106:
4102:
4092:
4089:
4088:
4086:
4082:
4076:
4073:
4072:
4070:
4066:
4063:
4061:
4057:
4047:
4044:
4042:
4039:
4038:
4036:
4032:
4029:
4027:
4023:
4013:
4010:
4009:
4007:
4003:
3997:
3994:
3993:
3991:
3987:
3984:
3982:
3978:
3975:
3973:
3969:
3955:
3952:
3950:
3947:
3946:
3944:
3940:
3937:
3935:
3931:
3921:
3918:
3916:
3913:
3909:
3906:
3904:
3901:
3900:
3899:
3895:
3892:
3891:
3889:
3885:
3882:
3880:
3876:
3866:
3863:
3862:
3860:
3856:
3853:
3851:
3847:
3837:
3834:
3833:
3831:
3827:
3824:
3822:
3818:
3808:
3805:
3804:
3802:
3798:
3795:
3793:
3789:
3786:
3784:
3780:
3766:
3763:
3761:
3758:
3757:
3755:
3751:
3748:
3746:
3742:
3739:
3737:
3733:
3729:
3725:
3718:
3713:
3711:
3706:
3704:
3699:
3698:
3695:
3679:
3676:
3674:
3671:
3670:
3668:
3664:
3658:
3655:
3653:
3650:
3648:
3645:
3643:
3640:
3638:
3635:
3633:
3630:
3628:
3625:
3623:
3620:
3618:
3615:
3613:
3610:
3608:
3604:
3598:
3597:
3594:
3591:
3589:
3586:
3584:
3581:
3579:
3572:
3570:
3567:
3565:
3562:
3561:
3559:
3555:
3552:
3550:
3545:
3541:Mitochondrial
3539:
3529:
3526:
3524:
3521:
3520:
3518:
3516:
3511:
3505:
3502:
3501:
3499:
3497:
3492:
3486:
3483:
3482:
3480:
3478:
3473:
3466:
3462:
3458:
3457:
3453:
3449:
3445:
3441:
3438:
3437:
3435:
3433:
3428:
3425:
3423:
3419:
3412:
3409:
3407:
3404:
3401:
3397:
3394:
3393:
3390:
3387:
3385:
3382:
3380:
3377:
3375:
3372:
3370:
3367:
3366:
3364:
3360:
3356:
3353:
3349:
3342:
3337:
3335:
3330:
3328:
3323:
3322:
3319:
3305:
3299:
3294:
3290:
3284:
3279:
3275:
3266:
3261:
3259:
3254:
3252:
3247:
3246:
3243:
3237:
3234:
3232:
3229:
3228:
3224:
3217:
3213:
3208:
3203:
3199:
3195:
3191:
3187:
3186:Diabetes Care
3183:
3178:
3174:
3170:
3166:
3162:
3156:
3152:
3148:
3144:
3140:
3136:
3132:
3128:
3124:
3118:
3114:
3110:
3105:
3100:
3095:
3090:
3086:
3082:
3079:(9): e12862.
3078:
3074:
3070:
3065:
3061:
3057:
3053:
3049:
3045:
3041:
3037:
3033:
3028:
3024:
3020:
3016:
3012:
3008:
3004:
3001:(6): 335–42.
3000:
2996:
2990:
2986:
2982:
2978:
2974:
2969:
2964:
2960:
2956:
2952:
2948:
2944:
2939:
2935:
2931:
2926:
2921:
2917:
2913:
2909:
2905:
2901:
2896:
2892:
2888:
2883:
2878:
2874:
2870:
2867:(4): 275–83.
2866:
2862:
2858:
2853:
2849:
2845:
2840:
2835:
2831:
2827:
2824:(5): 628–42.
2823:
2819:
2815:
2809:
2805:
2801:
2797:
2793:
2789:
2785:
2781:
2777:
2772:
2768:
2764:
2759:
2754:
2750:
2746:
2742:
2737:
2733:
2729:
2725:
2721:
2718:(6): 805–14.
2717:
2713:
2707:
2703:
2699:
2695:
2691:
2686:
2681:
2677:
2673:
2669:
2663:
2659:
2655:
2651:
2647:
2643:
2639:
2636:(3): 347–55.
2635:
2631:
2625:
2621:
2617:
2613:
2609:
2605:
2601:
2597:
2593:
2587:
2583:
2579:
2574:
2569:
2565:
2561:
2557:
2552:
2548:
2544:
2539:
2534:
2530:
2526:
2522:
2518:
2514:
2509:
2505:
2501:
2496:
2491:
2488:(3): 1274–8.
2487:
2483:
2479:
2474:
2470:
2466:
2461:
2456:
2453:(3): 929–37.
2452:
2448:
2444:
2439:
2438:
2433:
2431:
2428:
2427:
2425:
2424:public domain
2421:
2409:
2405:
2400:
2395:
2391:
2387:
2384:(8): 869–79.
2383:
2379:
2375:
2368:
2365:
2360:
2356:
2352:
2348:
2344:
2340:
2336:
2332:
2324:
2321:
2308:
2304:
2298:
2295:
2282:
2278:
2272:
2270:
2266:
2253:
2249:
2243:
2241:
2237:
2232:
2228:
2224:
2220:
2216:
2212:
2208:
2204:
2196:
2193:
2188:
2184:
2179:
2174:
2170:
2166:
2162:
2155:
2152:
2147:
2143:
2139:
2135:
2130:
2125:
2121:
2117:
2113:
2106:
2103:
2098:
2094:
2088:
2085:
2080:
2076:
2071:
2066:
2062:
2058:
2054:
2050:
2046:
2038:
2035:
2030:
2026:
2022:
2018:
2014:
2010:
2002:
1999:
1994:
1990:
1986:
1982:
1978:
1974:
1966:
1963:
1958:
1952:
1950:
1948:
1944:
1939:
1935:
1929:
1926:
1921:
1917:
1911:
1908:
1904:
1900:
1895:
1893:
1891:
1887:
1883:
1879:
1874:
1872:
1870:
1866:
1860:
1850:
1843:
1840:
1833:
1828:
1820:
1744:
1348:
1333:
1332:
1325:
1323:
1321:
1317:
1312:
1307:
1305:
1301:
1297:
1296:paraganglioma
1293:
1289:
1285:
1280:
1277:
1273:
1268:
1264:
1259:
1256:
1252:
1248:
1244:
1239:
1237:
1233:
1229:
1225:
1221:
1217:
1213:
1209:
1208:paraganglioma
1205:
1202:
1194:
1192:
1190:
1186:
1181:
1180:hydrogen bond
1177:
1173:
1169:
1165:
1161:
1157:
1153:
1152:deprotonation
1149:
1145:
1141:
1137:
1134:that anchors
1133:
1129:
1128:protein dimer
1121:
1119:
1117:
1113:
1109:
1105:
1101:
1097:
1096:transmembrane
1093:
1089:
1086:(Complex II)
1085:
1080:
1078:
1074:
1070:
1066:
1062:
1058:
1057:chromosomes 1
1054:
1050:
1046:
1042:
1041:chromosome 11
1038:
1030:
1028:
1026:
1022:
1018:
1014:
1010:
1006:
1003:
999:
996:(SDHD), is a
995:
991:
979:
974:
970:
966:
963:
959:
952:
950:
947:
943:
939:
935:
932:
928:
924:
917:
915:
909:
905:
902:
896:
894:
890:
886:
882:
876:
872:
869:
865:
858:
856:
850:
846:
843:
837:
835:
831:
827:
823:
817:
813:
810:
808:RefSeq (mRNA)
806:
799:
798:
793:
789:
786:
780:
779:
774:
770:
767:
765:
761:
754:
753:
748:
744:
741:
735:
734:
729:
725:
722:
720:
716:
709:
708:
703:
699:
696:
690:
689:
684:
680:
677:
675:
671:
668:
665:
663:
660:
656:
653:
649:
645:
638:
634:
629:
623:
620:
618:
615:
614:
612:
609:
608:
602:
599:
597:
594:
592:
591:mitochondrion
589:
587:
584:
582:
579:
577:
574:
573:
571:
568:
567:
561:
558:
556:
553:
551:
548:
546:
543:
541:
538:
536:
533:
532:
530:
527:
526:
523:
522:Gene ontology
519:
515:
503:
498:
495:
493:
489:
481:
476:
465:
461:
457:
453:
449:
445:
441:
437:
433:
429:
428:
425:
421:
416:
413:
403:
399:
395:
391:
387:
383:
379:
376:kidney tubule
375:
371:
367:
366:
363:
359:
354:
351:
350:
347:
345:
341:
339:
338:
334:
333:
330:
328:
324:
320:
316:
312:
304:
299:
295:
291:
286:
280:9|9 A5.3
276:
272:
265:
258:
252:
245:
237:
233:
229:
224:
220:
215:
211:
203:
198:
194:
190:
185:
175:
171:
164:
157:
151:
144:
140:
134:
130:
126:
121:
117:
112:
108:
104:
100:
96:
92:
88:
84:
80:
76:
72:
68:
60:
55:
48:
43:
38:
34:
29:
24:
21:
16:
5105:Translocases
5102:
5089:
5076:
5063:
5050:
5040:Transferases
5037:
5024:
4881:Binding site
4788:5α-reductase
4739:
4494:
3996:Beta-catenin
3599:
3588:Cytochrome c
3496:succinyl-CoA
3303:
3288:
3189:
3185:
3164:
3160:
3126:
3122:
3076:
3072:
3038:(5): 724–9.
3035:
3031:
2998:
2994:
2950:
2946:
2907:
2903:
2864:
2860:
2821:
2817:
2779:
2775:
2748:
2744:
2715:
2711:
2678:(1): 41–51.
2675:
2671:
2633:
2629:
2598:(9): 672–5.
2595:
2591:
2566:(3): 182–5.
2563:
2559:
2520:
2516:
2485:
2481:
2450:
2446:
2429:
2417:
2416:
2381:
2377:
2367:
2337:(3): 170–5.
2334:
2330:
2323:
2311:. Retrieved
2306:
2297:
2285:. Retrieved
2280:
2256:. Retrieved
2251:
2206:
2202:
2195:
2168:
2164:
2154:
2119:
2115:
2105:
2096:
2087:
2052:
2048:
2037:
2012:
2008:
2001:
1976:
1972:
1965:
1937:
1928:
1919:
1910:
1842:
1330:
1329:
1310:
1308:
1283:
1281:
1260:
1253:, SDHD, and
1240:
1235:
1231:
1223:
1219:
1215:
1200:
1198:
1125:
1094:. The other
1081:
1051:. There are
1034:
1019:in both the
1012:
1008:
1001:
993:
989:
988:
911:
889:NP_001263435
885:NP_001263433
881:NP_001263432
878:
852:
830:NM_001276506
826:NM_001276504
822:NM_001276503
819:
795:
776:
750:
731:
705:
686:
666:
661:
555:heme binding
430:right kidney
342:
335:
200:112,120,016
187:112,086,824
62:External IDs
20:
15:
4876:Active site
3605:synthesis:
3422:Anaplerotic
3271:PDB gallery
3167:(1): 43–8.
2910:(1): 62–6.
2751:(1): 7–11.
2303:"SDHD Gene"
1979:(1): 7–10.
1185:semiquinone
1098:subunit is
1077:amino acids
1053:pseudogenes
301:50,515,112
288:50,507,657
40:Identifiers
5148:Categories
5079:Isomerases
5053:Hydrolases
4920:Regulation
4319:DNA repair
4245:Cell cycle
3760:c-Sis/PDGF
3600:Coenzyme Q
3574:Coenzyme Q
3432:acetyl-CoA
3348:Metabolism
1905:, May 2017
1884:, May 2017
1861:References
1267:hamartomas
1172:ubiquinone
1140:ubiquinone
1136:Complex II
1049:base pairs
346:(ortholog)
83:HomoloGene
4958:EC number
3728:Oncogenes
3374:Aconitase
2630:Endocrine
1189:ubiquinol
1148:succinate
1146:oxidizes
1118:subunit.
1035:The SDHD
1031:Structure
914:NP_080124
893:NP_002993
855:NM_025848
834:NM_003002
652:Orthologs
91:GeneCards
5164:EC 1.3.5
4982:Kinetics
4906:Cofactor
4869:Activity
4641:acceptor
4594:acceptor
4525:Stathmin
4270:Cyclin E
4265:Cyclin D
3903:HER2/neu
3783:Receptor
3406:Fumarase
3216:20628086
3173:20205103
3143:19825962
3113:20877624
3073:PLOS ONE
3052:19393419
3015:19768395
2985:23793565
2977:16143825
2968:11139140
2934:19584903
2891:20208144
2848:19913121
2804:20549458
2796:12612654
2767:17298303
2732:20236688
2702:22888808
2694:19802898
2650:19399650
2620:24979281
2612:19343621
2582:20111059
2547:20153743
2504:19915015
2469:19546167
2408:26008905
2359:25057245
2351:24367056
2287:26 March
2258:26 March
2231:10657297
2187:16407191
2146:16697879
2138:15989954
2079:23965338
1901:–
1880:–
1176:carbonyl
1160:fumarate
1122:Function
1023:and the
962:Wikidata
631:Sources:
596:membrane
5138:Biology
5092:Ligases
4862:Enzymes
4712:Quinone
4515:c-Bcl-2
4323:Fanconi
4236:Nucleus
3578:(CoQ10)
3557:Primary
3413:and ETC
3355:enzymes
3207:2945168
3151:6225809
3104:2943476
3081:Bibcode
3060:5393912
3023:2265162
2925:2987152
2882:4619289
2839:2775832
2658:8986765
2538:3419008
2399:4495259
2313:30 July
2211:Bibcode
2203:Science
2070:4076475
2029:9533030
1993:1301144
1903:Ensembl
1882:Ensembl
1340:[[File:
1276:thyroid
1243:hypoxic
1154:at the
998:protein
764:UniProt
719:Ensembl
658:Species
637:QuickGO
321:pattern
179:11q23.1
79:1914175
47:Aliases
5124:Portal
5066:Lyases
4803:SRD5A3
4798:SRD5A2
4793:SRD5A1
4776:ACADSB
4752:1.3.99
4639:Oxygen
4305:p14arf
4218:Maspin
3898:c-ErbB
3736:Ligand
3647:COQ10B
3642:COQ10A
3214:
3204:
3171:
3149:
3141:
3111:
3101:
3058:
3050:
3021:
3013:
2983:
2975:
2965:
2932:
2922:
2889:
2879:
2846:
2836:
2802:
2794:
2765:
2730:
2700:
2692:
2656:
2648:
2618:
2610:
2580:
2545:
2535:
2502:
2467:
2406:
2396:
2357:
2349:
2277:"SDHD"
2229:
2185:
2144:
2136:
2077:
2067:
2027:
1991:
1272:breast
1255:SDHAF2
1106:/SDHD
1102:. The
1071:, and
1017:enzyme
948:search
946:PubMed
797:Q9CXV1
778:O14521
674:Entrez
492:BioGPS
372:rectum
71:602690
5018:Types
4781:ACADS
4708:1.3.5
4635:1.3.3
4584:1.3.1
4520:Notch
4439:c-Myc
4432:c-Jun
4427:c-Fos
4343:BRCA2
4338:BRCA1
4202:c-Src
4159:c-Raf
4149:c-Ras
4075:c-Akt
4046:SMAD4
4041:SMAD2
3949:c-Kit
3920:c-Ret
3915:c-Met
3908:Her 3
3836:PTCH1
3666:Other
3657:PDSS2
3652:PDSS1
3362:Cycle
3147:S2CID
3056:S2CID
3019:S2CID
2981:S2CID
2800:S2CID
2698:S2CID
2654:S2CID
2616:S2CID
2355:S2CID
2142:S2CID
1130:with
1108:dimer
1045:locus
707:66925
667:Mouse
662:Human
633:Amigo
344:Mouse
337:Human
284:Start
219:Mouse
183:Start
116:Human
87:37718
5110:list
5103:EC7
5097:list
5090:EC6
5084:list
5077:EC5
5071:list
5064:EC4
5058:list
5051:EC3
5045:list
5038:EC2
5032:list
5025:EC1
4740:SDHD
4735:SDHC
4730:SDHB
4725:SDHA
4592:NADP
4577:1.3)
4495:SDHD
4490:SDHB
4455:KLF6
4422:AP-1
4415:ONCO
4377:MDM2
4365:ONCO
4260:CDK4
4253:ONCO
4195:ONCO
4154:HRAS
4142:ONCO
4091:PTEN
4068:ONCO
3989:ONCO
3954:Flt3
3942:ONCO
3894:ErbB
3887:ONCO
3807:CDH1
3753:ONCO
3726:and
3637:COQ9
3632:COQ7
3627:COQ6
3622:COQ5
3617:COQ4
3612:COQ3
3607:COQ2
3463:and
3400:SDHA
3304:1zoy
3289:1zp0
3212:PMID
3169:PMID
3139:PMID
3109:PMID
3048:PMID
3011:PMID
2973:PMID
2930:PMID
2887:PMID
2844:PMID
2792:PMID
2763:PMID
2728:PMID
2690:PMID
2646:PMID
2608:PMID
2578:PMID
2543:PMID
2500:PMID
2465:PMID
2404:PMID
2347:PMID
2315:2018
2289:2015
2260:2015
2227:PMID
2183:PMID
2134:PMID
2116:Cell
2075:PMID
2025:PMID
1989:PMID
1832:edit
1819:edit
1311:SDHD
1284:SDHD
1274:and
1251:SDHC
1247:SDHB
1236:SDHD
1232:SDHB
1224:SDHC
1220:SDHB
1216:SDHD
1204:gene
1201:SDHD
1164:SDHB
1150:via
1144:SDHA
1132:SDHC
1116:SDHA
1112:SDHB
1104:SDHC
1100:SDHC
1037:gene
1013:PGL1
1011:and
1005:gene
1002:SDHD
688:6392
327:Bgee
275:Band
236:Chr.
174:Band
133:Chr.
95:SDHD
67:OMIM
54:SDHD
26:SDHD
4588:NAD
4448:TSP
4393:VHL
4386:TSP
4372:CBL
4331:TSP
4301:p16
4296:WT1
4291:pRb
4286:p53
4279:TSP
4211:TSP
4168:TSP
4113:TSP
4084:TSP
4034:TSP
4012:APC
4005:TSP
3858:TSP
3829:TSP
3800:TSP
3765:HGF
3513:to
3494:to
3475:to
3430:to
3202:PMC
3194:doi
3131:doi
3099:PMC
3089:doi
3040:doi
3036:140
3003:doi
2963:PMC
2955:doi
2920:PMC
2912:doi
2877:PMC
2869:doi
2834:PMC
2826:doi
2784:doi
2753:doi
2720:doi
2680:doi
2638:doi
2600:doi
2568:doi
2533:PMC
2525:doi
2490:doi
2455:doi
2394:PMC
2386:doi
2382:134
2339:doi
2219:doi
2207:287
2173:doi
2169:281
2124:doi
2120:121
2065:PMC
2057:doi
2053:113
2017:doi
1981:doi
1318:or
1298:or
1214:in
1156:FAD
1043:at
1009:PGL
500:n/a
297:End
196:End
99:OMA
75:MGI
5150::
4710::
4637::
4586::
4575:EC
4569::
3602:10
3576:10
3452:E3
3450:,
3448:E2
3446:,
3444:E1
3350::
3210:.
3200:.
3190:33
3188:.
3184:.
3165:61
3163:.
3145:.
3137:.
3127:15
3125:.
3107:.
3097:.
3087:.
3075:.
3071:.
3054:.
3046:.
3034:.
3017:.
3009:.
2999:19
2997:.
2979:.
2971:.
2961:.
2951:62
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2945:.
2928:.
2918:.
2908:18
2906:.
2902:.
2885:.
2875:.
2865:32
2863:.
2859:.
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2832:.
2822:85
2820:.
2816:.
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2790:.
2778:.
2761:.
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2716:41
2714:.
2696:.
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2670:.
2652:.
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2606:.
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2181:.
2167:.
2163:.
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2132:.
2118:.
2114:.
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2073:.
2063:.
2051:.
2047:.
2023:.
2013:79
2011:.
1987:.
1975:.
1946:^
1936:.
1918:.
1889:^
1868:^
1821:]]
1322:.
1249:,
1210:.
1191:.
1079:.
1073:18
1067:,
1063:,
1059:,
635:/
303:bp
290:bp
202:bp
189:bp
97:;
93::
89:;
85::
81:;
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73:;
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5108:(
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5030:(
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2704:.
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2549:.
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2471:.
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2426:.
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2221::
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2099:.
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2031:.
2019::
1995:.
1983::
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1959:.
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1922:.
1851:.
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1646:]
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1631:]
1626:]
1621:]
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1436:]
1431:]
1426:]
1421:]
1069:7
1065:3
1061:2
221:)
118:)
101::
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