196:
920:
116:
1172:. Abnormalities in mitochondrial energy generation result in neurodegenerative disorders like Leigh syndrome, which is characterized by an onset of symptoms between 12 months and three years of age. The symptoms frequently present themselves following a viral infection and include movement disorders and
2427:
Tanaka M, Cabrera VM, González AM, Larruga JM, Takeyasu T, Fuku N, Guo LJ, Hirose R, Fujita Y, Kurata M, Shinoda K, Umetsu K, Yamada Y, Oshida Y, Sato Y, Hattori N, Mizuno Y, Arai Y, Hirose N, Ohta S, Ogawa O, Tanaka Y, Kawamori R, Shamoto-Nagai M, Maruyama W, Shimokata H, Suzuki R, Shimodaira H
1034:
domain for the peripheral arm that includes all the known redox centres and the NADH binding site. The MT-ND3 product and the rest of the mitochondrially encoded subunits are the most hydrophobic of the subunits of
Complex I and form the core of the transmembrane region.
1733:
Leshinsky-Silver E, Lev D, Tzofi-Berman Z, Cohen S, Saada A, Yanoov-Sharav M, Gilad E, Lerman-Sagie T (August 2005). "Fulminant neurological deterioration in a neonate with Leigh syndrome due to a maternally transmitted missense mutation in the mitochondrial ND3 gene".
963:
1453:
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).
2354:
Coble MD, Just RS, O'Callaghan JE, Letmanyi IH, Peterson CT, Irwin JA, Parsons TJ (June 2004). "Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in
Caucasians".
1806:
Grosso S, Carluccio MA, Cardaioli E, Cerase A, Malandrini A, Romano C, Federico A, Dotti MT (March 2017). "Complex I deficiency related to T10158C mutation ND3 gene: A further definition of the clinical spectrum".
1051:
are alternative explanations for maintenance of the functionality of the ND3 reading frame in birds possessing the one-nucleotide insertion. This extra nucleotide feature suggests that turtles might be related to
2102:
Silva WA, Bonatto SL, Holanda AJ, Ribeiro-Dos-Santos AK, Paixão BM, Goldman GH, Abe-Sandes K, Rodriguez-Delfin L, Barbosa M, Paçó-Larson ML, Petzl-Erler ML, Valente V, Santos SE, Zago MA (July 2002).
991:
from base pair 10,059 to 10,404. The MT-ND3 gene produces a 13 kDa protein composed of 115 amino acids. MT-ND3 is one of seven mitochondrial genes encoding subunits of the enzyme
2557:
Achilli A, Rengo C, Battaglia V, Pala M, Olivieri A, Fornarino S, Magri C, Scozzari R, Babudri N, Santachiara-Benerecetti AS, Bandelt HJ, Semino O, Torroni A (May 2005).
2714:
2392:
Crimi M, Papadimitriou A, Galbiati S, Palamidou P, Fortunato F, Bordoni A, Papandreou U, Papadimitriou D, Hadjigeorgiou GM, Drogari E, Bresolin N, Comi GP (May 2004).
1402:
La Morgia C, Caporali L, Gandini F, Olivieri A, Toni F, Nassetti S, Brunetto D, Stipa C, Scaduto C, Parmeggiani A, Tonon C, Lodi R, Torroni A, Carelli V (May 2014).
2645:
Friedlaender J, Schurr T, Gentz F, Koki G, Friedlaender F, Horvat G, Babb P, Cerchio S, Kaestle F, Schanfield M, Deka R, Yanagihara R, Merriwether DA (June 2005).
1060:
studies. The absence of the extra nucleotide in crocodilians and some birds and turtles might also indicate that the corresponding taxa have lost this feature.
2180:
Mishmar D, Ruiz-Pesini E, Golik P, Macaulay V, Clark AG, Hosseini S, Brandon M, Easley K, Chen E, Brown MD, Sukernik RI, Olckers A, Wallace DC (January 2003).
2514:
Starikovskaya EB, Sukernik RI, Derbeneva OA, Volodko NV, Ruiz-Pesini E, Torroni A, Brown MD, Lott MT, Hosseini SH, Huoponen K, Wallace DC (January 2005).
3195:
666:
647:
77:
3190:
3281:
2863:
2949:
1201:
971:
2061:"Reduced-median-network analysis of complete mitochondrial DNA coding-region sequences for the major African, Asian, and European haplogroups"
1304:
2758:
1286:
2707:
1765:"Long survival in patients with leigh syndrome and the m.10191T>C mutation in MT-ND3 : a case report and review of the literature"
2516:"Mitochondrial DNA diversity in indigenous populations of the southern extent of Siberia, and the origins of Native American haplogroups"
195:
3507:
3049:
2984:
2473:"Phylogeny of mitochondrial DNA macrohaplogroup N in India, based on complete sequencing: implications for the peopling of South Asia"
2994:
2819:
1353:
2059:
Herrnstadt C, Elson JL, Fahy E, Preston G, Turnbull DM, Anderson C, Ghosh SS, Olefsky JM, Beal MF, Davis RE, Howell N (May 2002).
3320:
3118:
2753:
1325:
115:
1926:
Ingman M, Kaessmann H, Pääbo S, Gyllensten U (December 2000). "Mitochondrial genome variation and the origin of modern humans".
1221:
3487:
3315:
2979:
2700:
1273:
1252:
992:
951:
860:
1376:. In Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJ, Bird TD, Dolan CR, Fong CT, Smith RJ, Stephens K (eds.).
3310:
3096:
3054:
1269:
1043:
In the MT-ND3 gene from many species of birds and turtles there is an extra nucleotide that is not translated to protein.
867:
2687:
2471:
Palanichamy MG, Sun C, Agrawal S, Bandelt HJ, Kong QP, Khan F, Wang CY, Chaudhuri TK, Palla V, Zhang YP (December 2004).
1248:
2900:
2842:
2732:
955:
57:
2954:
2147:"Asian-specific mtDNA backgrounds associated with the primary G11778A mutation of Leber's hereditary optic neuropathy"
2104:"Mitochondrial genome diversity of Native Americans supports a single early entry of founder populations into America"
1844:
Torroni A, Achilli A, Macaulay V, Richards M, Bandelt HJ (June 2006). "Harvesting the fruit of the human mtDNA tree".
1044:
3128:
3123:
2927:
2793:
1704:
1404:"Association of the mtDNA m.4171C>A/MT-ND1 mutation with both optic neuropathy and bilateral brainstem lesions"
2853:
2602:"Phylogeny and antiquity of M macrohaplogroup inferred from complete mt DNA sequence of Indian specific lineages"
1588:"Phylogenomic analyses support the position of turtles as the sister group of birds and crocodiles (Archosauria)"
129:
3044:
2922:
959:
711:
65:
2321:"Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J"
3091:
2878:
692:
1112:). The flow of electrons changes the redox state of the protein, resulting in a conformational change and p
919:
3086:
2743:
1639:"More than 1000 ultraconserved elements provide evidence that turtles are the sister group of archosaurs"
3460:
3200:
3076:
2912:
2890:
1173:
1145:
1093:
1875:"Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs"
3301:
3207:
3064:
3013:
2959:
2193:
1935:
1886:
943:
44:
1521:
1116:
shift of the ionizable side chain, which pumps four hydrogen ions out of the mitochondrial matrix.
1101:
1057:
967:
2235:"Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines"
1023:, this enzyme is the largest of the respiratory complexes. The structure is L-shaped with a long,
3231:
2999:
2873:
2394:"A new mitochondrial DNA mutation in ND3 gene causing severe Leigh syndrome with early lethality"
2380:
1959:
1832:
1089:
89:
843:
1188:. Roughly half of affected patients die of respiratory or cardiac failure by the age of three.
3512:
3221:
3024:
2805:
2668:
2633:
2588:
2545:
2502:
2459:
2415:
2372:
2342:
2307:
2264:
2221:
2168:
2133:
2090:
2047:
2002:
1951:
1914:
1861:
1824:
1794:
1751:
1668:
1619:
1568:
1485:
1435:
1381:
1349:
1185:
1077:
988:
37:
3034:
2827:
2658:
2623:
2613:
2578:
2570:
2535:
2527:
2492:
2484:
2449:
2441:
2405:
2364:
2332:
2297:
2289:
2254:
2246:
2211:
2201:
2158:
2123:
2115:
2080:
2072:
2037:
2027:
1992:
1984:
1943:
1904:
1894:
1853:
1816:
1784:
1776:
1743:
1658:
1650:
1609:
1599:
1558:
1475:
1467:
1425:
1415:
288:
219:
186:
141:
2939:
2832:
1637:
Crawford NG, Faircloth BC, McCormack JE, Brumfield RT, Winker K, Glenn TC (October 2012).
1563:
1546:
1193:
263:
1124:
Pathogenic variants of the mitochondrial gene MT-ND3 are known to cause mtDNA-associated
1072:
that is believed to belong to the minimal assembly of core proteins required to catalyze
85:
2197:
1939:
1890:
2628:
2601:
2583:
2558:
2540:
2515:
2497:
2472:
2410:
2393:
2302:
2277:
1997:
1972:
1789:
1764:
1663:
1638:
1614:
1587:
1480:
1455:
1430:
1403:
1209:
1189:
1125:
2454:
2429:
2259:
2234:
2216:
2181:
2128:
2103:
2085:
2060:
1547:"An extra nucleotide is not translated in mitochondrial ND3 of some birds and turtles"
581:
576:
571:
566:
561:
556:
540:
535:
530:
525:
520:
515:
510:
505:
489:
484:
479:
3501:
2723:
2531:
2278:"Phylogeny of east Asian mitochondrial DNA lineages inferred from complete sequences"
2042:
2015:
1909:
1874:
1503:
1456:"Integration of cardiac proteome biology and medicine by a specialized knowledgebase"
1027:
466:
1963:
1200:
genes have been associated with a variety of neurodegenerative disorders, including
16:
Mitochondrial protein-coding gene whose product is involved in the respiratory chain
3356:
3331:
2989:
2868:
2781:
2384:
1836:
1820:
281:
106:
69:
1471:
93:
1309:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1291:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1053:
1048:
1031:
1024:
2186:
Proceedings of the
National Academy of Sciences of the United States of America
1879:
Proceedings of the
National Academy of Sciences of the United States of America
1747:
364:
3108:
2770:
2368:
1857:
1181:
1081:
203:
123:
73:
48:, MTMT-NADH dehydrogenase, subunit 3 (complex I), NADH dehydrogenase subunit 3
2145:
Sudoyo H, Suryadi H, Lertrit P, Pramoonjago P, Lyrawati D, Marzuki S (2003).
1780:
1420:
1373:
1224:
including 2 co-complex interactions. MT-ND3 appears to interact with APP and
1104:
in the prosthetic arm and finally to coenzyme Q10 (CoQ), which is reduced to
3039:
2663:
2646:
2337:
2320:
2206:
1604:
1197:
1192:
is a maternally inherited disorder and its diagnosis is established through
1177:
1105:
1069:
1020:
611:
424:
302:
247:
234:
169:
156:
81:
2672:
2637:
2618:
2592:
2549:
2506:
2463:
2419:
2376:
2346:
2311:
2268:
2225:
2172:
2137:
2094:
2051:
2032:
2006:
1955:
1865:
1828:
1798:
1755:
1672:
1654:
1623:
1572:
1489:
1439:
1385:
2430:"Mitochondrial genome variation in eastern Asia and the peopling of Japan"
2163:
2146:
1918:
1899:
1763:
Levy RJ, Ríos PG, Akman HO, Sciacco M, Vivo DC, DiMauro S (October 2014).
964:
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes
931:
is one of the seven NADH dehydrogenase mitochondrial genes (yellow boxes).
907:
902:
3185:
3059:
2692:
1686:
891:
756:
737:
2600:
Rajkumar R, Banerjee J, Gunturi HB, Trivedi R, Kashyap VK (April 2005).
1687:"MT-ND3 - NADH-ubiquinone oxidoreductase chain 3 - Homo sapiens (Human)"
3345:
3340:
3260:
2445:
2276:
Kong QP, Yao YG, Sun C, Bandelt HJ, Zhu CL, Zhang YP (September 2003).
2016:"Major genomic mitochondrial lineages delineate early human expansions"
1129:
1008:
723:
678:
2250:
3450:
3445:
3415:
3410:
3290:
3270:
3265:
3255:
3245:
3240:
1947:
1225:
1169:
1165:
1161:
1157:
1153:
1149:
1133:
1016:
1012:
1004:
1000:
996:
875:
633:
2014:
Maca-Meyer N, González AM, Larruga JM, Flores C, Cabrera VM (2003).
1873:
Horai S, Hayasaka K, Kondo R, Tsugane K, Takahata N (January 1995).
2574:
2488:
2293:
2119:
2076:
1988:
1204:(LHON), mitochondrial encephalomyopathy with stroke-like episodes (
1196:
of the aforementioned mitochondrial genes, including MT-ND3. These
3470:
3465:
3455:
3440:
3435:
3430:
3425:
3420:
3405:
3400:
3395:
3390:
3385:
3380:
3375:
3370:
3365:
3161:
3148:
1205:
1141:
1137:
596:
592:
1085:
1073:
939:
61:
2696:
2647:"Expanding Southwest Pacific mitochondrial haplogroups P and Q"
1346:
Fundamentals of biochemistry : life at the molecular level
1344:
Donald Voet; Judith G. Voet; Charlotte W. Pratt (2013). "18".
2182:"Natural selection shaped regional mtDNA variation in humans"
2559:"Saami and Berbers--an unexpected mitochondrial DNA link"
1068:
The MT-ND3 product is a subunit of the respiratory chain
271:
1508:
Cardiac
Organellar Protein Atlas Knowledgebase (COPaKB)
2688:
Mass spectrometry characterization of MT-ND3 at COPaKB
1545:
Mindell DP, Sorenson MD, Dimcheff DE (November 1998).
1374:"Mitochondrial DNA-Associated Leigh Syndrome and NARP"
1348:(4th ed.). Hoboken, NJ: Wiley. pp. 581–620.
1088:
binds to
Complex I and transfers two electrons to the
1586:
Chiari Y, Cahais V, Galtier N, Delsuc F (July 2012).
436:
1705:"5 binary interactions found for search term MT-ND3"
1100:. The electrons are transferred through a series of
582:
mitochondrial electron transport, NADH to ubiquinone
3354:
3329:
3299:
3279:
3229:
3220:
3174:
3137:
3105:
3073:
3021:
3012:
2968:
2936:
2909:
2887:
2850:
2841:
2818:
2790:
2767:
2740:
2731:
2319:Moilanen JS, Finnila S, Majamaa K (December 2003).
1736:
1380:. Seattle (WA): University of Washington, Seattle.
836:
817:
793:
774:
1326:"Entrez Gene: MT-ND3 NADH dehydrogenase subunit 3"
1265:
1263:
1261:
1244:
1242:
1240:
577:mitochondrial respiratory chain complex I assembly
218:
140:
958:and is the largest of the five complexes of the
1971:Finnilä S, Lehtonen MS, Majamaa K (June 2001).
1397:
1395:
950:(ND3) protein. The ND3 protein is a subunit of
1270:GRCm38: Ensembl release 89: ENSMUSG00000064360
2708:
1367:
1365:
1320:
1318:
8:
567:cellular response to glucocorticoid stimulus
1249:GRCh38: Ensembl release 89: ENSG00000198840
3226:
3196:Mitochondrial permeability transition pore
3178:
3018:
2847:
2737:
2715:
2701:
2693:
1691:UniProt.org: a hub for protein information
607:
462:
259:
181:
101:
2662:
2627:
2617:
2582:
2539:
2496:
2453:
2409:
2336:
2301:
2258:
2215:
2205:
2162:
2127:
2084:
2041:
2031:
1996:
1973:"Phylogenetic network for European mtDNA"
1908:
1898:
1788:
1662:
1613:
1603:
1562:
1522:"Complete mitochondrial genome sequences"
1479:
1429:
1419:
1339:
1337:
1335:
962:. Variants of MT-ND3 are associated with
516:mitochondrial respiratory chain complex I
3191:Mitochondrial membrane transport protein
1504:"NADH-ubiquinone oxidoreductase chain 3"
927:gene in the human mitochondrial genome.
918:
490:NADH dehydrogenase (ubiquinone) activity
2357:International Journal of Legal Medicine
1236:
1220:MT-ND3 has been shown to have 5 binary
2950:Cholesterol side-chain cleavage enzyme
18:
1709:IntAct Molecular Interaction Database
1564:10.1093/oxfordjournals.molbev.a025884
223:
207:
202:
145:
127:
122:
7:
2233:Ingman M, Gyllensten U (July 2003).
2864:Coenzyme Q – cytochrome c reductase
1372:Thorburn DR, Rahman S (1993–2015).
1202:Leber's hereditary optic neuropathy
972:Leber's hereditary optic neuropathy
3050:Oxoglutarate dehydrogenase complex
2985:Glycerol-3-phosphate dehydrogenase
2563:American Journal of Human Genetics
2477:American Journal of Human Genetics
2411:10.1203/01.PDR.0000117844.73436.68
2282:American Journal of Human Genetics
2108:American Journal of Human Genetics
2065:American Journal of Human Genetics
1977:American Journal of Human Genetics
833:
814:
790:
771:
747:
728:
702:
683:
657:
638:
441:
359:
297:
276:
14:
2995:Carnitine palmitoyltransferase II
1096:(FMN) prosthetic arm to form FMNH
3119:Carbamoyl phosphate synthetase I
2759:Long-chain-fatty-acid—CoA ligase
2754:Carnitine palmitoyltransferase I
2532:10.1046/j.1529-8817.2003.00127.x
194:
114:
2980:Glutamate aspartate transporter
2651:Molecular Biology and Evolution
2325:Molecular Biology and Evolution
1551:Molecular Biology and Evolution
1208:) and the previously mentioned
993:NADH dehydrogenase (ubiquinone)
952:NADH dehydrogenase (ubiquinone)
3097:Pyruvate dehydrogenase complex
3055:Succinyl coenzyme A synthetase
1821:10.1016/j.braindev.2016.09.013
506:integral component of membrane
425:More reference expression data
1:
1472:10.1161/CIRCRESAHA.113.301151
1039:Untranslated extra nucleotide
329:muscle layer of sigmoid colon
2901:Dihydroorotate dehydrogenase
1222:protein-protein interactions
956:mitochondrial inner membrane
572:response to oxidative stress
511:mitochondrial inner membrane
2955:Steroid 11-beta-hydroxylase
1102:iron-sulfur (Fe-S) clusters
1084:(coenzyme Q10). Initially,
1045:Translational frameshifting
987:MT-ND3 is located in human
562:response to light intensity
337:subcutaneous adipose tissue
3529:
3129:N-Acetylglutamate synthase
3124:Ornithine transcarbamylase
2928:Glycerol phosphate shuttle
2794:monoamine neurotransmitter
1769:Journal of Child Neurology
1748:10.1016/j.bbrc.2005.06.134
954:, which is located in the
541:NADH dehydrogenase complex
3508:Human mitochondrial genes
3483:
3181:
3157:
2854:oxidative phosphorylation
2369:10.1007/s00414-004-0427-6
2151:Journal of Human Genetics
1858:10.1016/j.tig.2006.04.001
1693:. The UniProt Consortium.
1305:"Mouse PubMed Reference:"
1287:"Human PubMed Reference:"
906:
901:
897:
890:
874:
855:
840:
821:
810:
797:
778:
767:
754:
750:
735:
731:
722:
709:
705:
690:
686:
677:
664:
660:
645:
641:
632:
617:
610:
606:
590:
465:
461:
449:
444:
435:
422:
391:quadriceps femoris muscle
371:
362:
309:
300:
270:
262:
258:
241:
228:
214:
209:Mitochondrial DNA (mouse)
193:
184:
180:
163:
150:
136:
130:Mitochondrial DNA (human)
113:
104:
100:
55:
52:
42:
35:
30:
26:
21:
3045:Isocitrate dehydrogenase
2923:Malate-aspartate shuttle
2606:BMC Evolutionary Biology
2520:Annals of Human Genetics
1781:10.1177/0883073813506783
1526:megasun.bch.umontreal.ca
1421:10.1186/1471-2377-14-116
960:electron transport chain
3092:Glutamate dehydrogenase
2879:Succinate dehydrogenase
2207:10.1073/pnas.0136972100
1809:Brain & Development
1605:10.1186/1741-7007-10-65
536:mitochondrial membranes
480:oxidoreductase activity
3488:mitochondrial diseases
3087:Aspartate transaminase
2744:fatty acid degradation
2619:10.1186/1471-2148-5-26
2033:10.1186/1471-2156-2-13
1655:10.1098/rsbl.2012.0331
932:
407:neural layer of retina
399:superior frontal gyrus
3201:Mitochondrial carrier
3077:anaplerotic reactions
2913:mitochondrial shuttle
2891:pyrimidine metabolism
2664:10.1093/molbev/msi142
2338:10.1093/molbev/msg230
2164:10.1007/s100380200091
1900:10.1073/pnas.92.2.532
1174:peripheral neuropathy
1128:, as are variants of
1120:Clinical significance
1094:flavin mononucleotide
922:
868:Chr M: 0.01 – 0.01 Mb
861:Chr M: 0.01 – 0.01 Mb
3208:Translocator protein
3065:Malate dehydrogenase
2960:Aldosterone synthase
1460:Circulation Research
1076:dehydrogenation and
948:NADH dehydrogenase 3
944:mitochondrial genome
2820:Intermembrane space
2198:2003PNAS..100..171M
1940:2000Natur.408..708I
1891:1995PNAS...92..532H
1058:molecular phylogeny
557:response to hormone
395:islet of Langerhans
345:left adrenal cortex
341:right adrenal gland
3175:Other/to be sorted
3140:alcohol metabolism
3000:Uncoupling protein
2874:NADH dehydrogenase
2446:10.1101/gr.2286304
2398:Pediatric Research
1846:Trends in Genetics
1090:isoalloxazine ring
1056:, as evidenced by
933:
712:ENSMUSG00000064360
550:Biological process
499:Cellular component
473:Molecular function
379:hippocampus proper
313:right uterine tube
3495:
3494:
3479:
3478:
3222:Mitochondrial DNA
3216:
3215:
3170:
3169:
3025:citric acid cycle
3008:
3007:
2814:
2813:
2806:Monoamine oxidase
2251:10.1101/gr.686603
1186:cerebellar ataxia
1078:electron transfer
989:mitochondrial DNA
917:
916:
913:
912:
886:
885:
851:
850:
830:
829:
806:
805:
787:
786:
763:
762:
744:
743:
718:
717:
699:
698:
673:
672:
654:
653:
602:
601:
457:
456:
453:
452:
431:
430:
418:
417:
356:
355:
333:lactiferous gland
254:
253:
176:
175:
3520:
3359:
3334:
3304:
3284:
3234:
3227:
3179:
3142:
3112:
3080:
3035:Citrate synthase
3028:
3019:
2973:
2943:
2916:
2894:
2857:
2848:
2828:Adenylate kinase
2799:
2775:
2747:
2738:
2717:
2710:
2703:
2694:
2676:
2666:
2641:
2631:
2621:
2596:
2586:
2553:
2543:
2510:
2500:
2467:
2457:
2440:(10A): 1832–50.
2428:(October 2004).
2423:
2413:
2388:
2350:
2340:
2315:
2305:
2272:
2262:
2229:
2219:
2209:
2176:
2166:
2141:
2131:
2098:
2088:
2055:
2045:
2035:
2010:
2000:
1967:
1948:10.1038/35047064
1934:(6813): 708–13.
1922:
1912:
1902:
1869:
1840:
1802:
1792:
1775:(10): NP105–10.
1759:
1720:
1719:
1717:
1716:
1701:
1695:
1694:
1683:
1677:
1676:
1666:
1634:
1628:
1627:
1617:
1607:
1583:
1577:
1576:
1566:
1542:
1536:
1535:
1533:
1532:
1518:
1512:
1511:
1500:
1494:
1493:
1483:
1450:
1444:
1443:
1433:
1423:
1399:
1390:
1389:
1369:
1360:
1359:
1341:
1330:
1329:
1322:
1313:
1312:
1301:
1295:
1294:
1283:
1277:
1267:
1256:
1246:
1019:. Also known as
995:, together with
983:General features
968:Leigh's syndrome
923:Location of the
899:
898:
870:
863:
846:
834:
825:
815:
811:RefSeq (protein)
801:
791:
782:
772:
748:
729:
703:
684:
658:
639:
608:
463:
442:
427:
367:
365:Top expressed in
360:
305:
303:Top expressed in
298:
277:
260:
250:
237:
226:
210:
198:
182:
172:
159:
148:
132:
118:
102:
96:
47:
40:
19:
3528:
3527:
3523:
3522:
3521:
3519:
3518:
3517:
3498:
3497:
3496:
3491:
3475:
3355:
3350:
3330:
3325:
3300:
3295:
3280:
3275:
3230:
3212:
3166:
3153:
3138:
3133:
3106:
3101:
3074:
3069:
3022:
3004:
2969:
2964:
2940:steroidogenesis
2937:
2932:
2910:
2905:
2888:
2883:
2851:
2837:
2833:Creatine kinase
2810:
2796:
2791:
2786:
2768:
2763:
2741:
2727:
2721:
2684:
2679:
2644:
2599:
2556:
2526:(Pt 1): 67–89.
2513:
2470:
2434:Genome Research
2426:
2391:
2353:
2331:(12): 2132–42.
2318:
2275:
2239:Genome Research
2232:
2179:
2157:(11): 594–604.
2144:
2101:
2058:
2013:
1970:
1925:
1872:
1843:
1805:
1762:
1732:
1728:
1726:Further reading
1723:
1714:
1712:
1703:
1702:
1698:
1685:
1684:
1680:
1643:Biology Letters
1636:
1635:
1631:
1585:
1584:
1580:
1557:(11): 1568–71.
1544:
1543:
1539:
1530:
1528:
1520:
1519:
1515:
1502:
1501:
1497:
1452:
1451:
1447:
1401:
1400:
1393:
1371:
1370:
1363:
1356:
1343:
1342:
1333:
1324:
1323:
1316:
1303:
1302:
1298:
1285:
1284:
1280:
1268:
1259:
1247:
1238:
1234:
1218:
1194:genetic testing
1122:
1111:
1099:
1066:
1041:
985:
980:
946:coding for the
908:View/Edit Mouse
903:View/Edit Human
866:
859:
856:Location (UCSC)
842:
823:
799:
780:
693:ENSG00000198840
586:
545:
494:
485:protein binding
423:
414:
411:muscle of thigh
409:
405:
401:
397:
393:
389:
385:
381:
377:
363:
352:
347:
343:
339:
335:
331:
327:
325:Brodmann area 9
323:
319:
315:
301:
245:
232:
224:
208:
185:Gene location (
167:
154:
146:
128:
105:Gene location (
94:ND3 - orthologs
56:
43:
36:
17:
12:
11:
5:
3526:
3524:
3516:
3515:
3510:
3500:
3499:
3493:
3492:
3484:
3481:
3480:
3477:
3476:
3474:
3473:
3468:
3463:
3458:
3453:
3448:
3443:
3438:
3433:
3428:
3423:
3418:
3413:
3408:
3403:
3398:
3393:
3388:
3383:
3378:
3373:
3368:
3362:
3360:
3352:
3351:
3349:
3348:
3343:
3337:
3335:
3327:
3326:
3324:
3323:
3318:
3313:
3307:
3305:
3297:
3296:
3294:
3293:
3287:
3285:
3277:
3276:
3274:
3273:
3268:
3263:
3258:
3253:
3248:
3243:
3237:
3235:
3224:
3218:
3217:
3214:
3213:
3211:
3210:
3205:
3204:
3203:
3198:
3188:
3182:
3176:
3172:
3171:
3168:
3167:
3165:
3164:
3158:
3155:
3154:
3152:
3151:
3145:
3143:
3135:
3134:
3132:
3131:
3126:
3121:
3115:
3113:
3103:
3102:
3100:
3099:
3094:
3089:
3083:
3081:
3071:
3070:
3068:
3067:
3062:
3057:
3052:
3047:
3042:
3037:
3031:
3029:
3016:
3010:
3009:
3006:
3005:
3003:
3002:
2997:
2992:
2987:
2982:
2976:
2974:
2966:
2965:
2963:
2962:
2957:
2952:
2946:
2944:
2934:
2933:
2931:
2930:
2925:
2919:
2917:
2907:
2906:
2904:
2903:
2897:
2895:
2885:
2884:
2882:
2881:
2876:
2871:
2866:
2860:
2858:
2845:
2843:Inner membrane
2839:
2838:
2836:
2835:
2830:
2824:
2822:
2816:
2815:
2812:
2811:
2809:
2808:
2802:
2800:
2788:
2787:
2785:
2784:
2778:
2776:
2765:
2764:
2762:
2761:
2756:
2750:
2748:
2735:
2733:Outer membrane
2729:
2728:
2722:
2720:
2719:
2712:
2705:
2697:
2691:
2690:
2683:
2682:External links
2680:
2678:
2677:
2657:(6): 1506–17.
2642:
2597:
2575:10.1086/430073
2554:
2511:
2489:10.1086/425871
2468:
2424:
2389:
2351:
2316:
2294:10.1086/377718
2273:
2230:
2177:
2142:
2120:10.1086/341358
2099:
2077:10.1086/339933
2071:(5): 1152–71.
2056:
2011:
1989:10.1086/320591
1983:(6): 1475–84.
1968:
1923:
1870:
1841:
1815:(3): 261–265.
1803:
1760:
1729:
1727:
1724:
1722:
1721:
1696:
1678:
1629:
1578:
1537:
1513:
1495:
1466:(9): 1043–53.
1445:
1391:
1361:
1354:
1331:
1314:
1296:
1278:
1257:
1235:
1233:
1230:
1217:
1214:
1210:Leigh syndrome
1190:Leigh syndrome
1126:Leigh syndrome
1121:
1118:
1109:
1097:
1065:
1062:
1040:
1037:
984:
981:
979:
976:
915:
914:
911:
910:
905:
895:
894:
888:
887:
884:
883:
881:
879:
872:
871:
864:
857:
853:
852:
849:
848:
838:
837:
831:
828:
827:
819:
818:
812:
808:
807:
804:
803:
795:
794:
788:
785:
784:
776:
775:
769:
765:
764:
761:
760:
752:
751:
745:
742:
741:
733:
732:
726:
720:
719:
716:
715:
707:
706:
700:
697:
696:
688:
687:
681:
675:
674:
671:
670:
662:
661:
655:
652:
651:
643:
642:
636:
630:
629:
624:
619:
615:
614:
604:
603:
600:
599:
588:
587:
585:
584:
579:
574:
569:
564:
559:
553:
551:
547:
546:
544:
543:
538:
533:
528:
523:
518:
513:
508:
502:
500:
496:
495:
493:
492:
487:
482:
476:
474:
470:
469:
459:
458:
455:
454:
451:
450:
447:
446:
439:
433:
432:
429:
428:
420:
419:
416:
415:
413:
412:
408:
404:
400:
396:
392:
388:
384:
383:olfactory bulb
380:
376:
372:
369:
368:
357:
354:
353:
351:
350:
346:
342:
338:
334:
330:
326:
322:
318:
314:
310:
307:
306:
294:
293:
285:
274:
268:
267:
264:RNA expression
256:
255:
252:
251:
243:
239:
238:
230:
227:
222:
216:
215:
212:
211:
206:
200:
199:
191:
190:
178:
177:
174:
173:
165:
161:
160:
152:
149:
144:
138:
137:
134:
133:
126:
120:
119:
111:
110:
98:
97:
54:
50:
49:
41:
33:
32:
28:
27:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
3525:
3514:
3511:
3509:
3506:
3505:
3503:
3490:
3489:
3482:
3472:
3469:
3467:
3464:
3462:
3459:
3457:
3454:
3452:
3449:
3447:
3444:
3442:
3439:
3437:
3434:
3432:
3429:
3427:
3424:
3422:
3419:
3417:
3414:
3412:
3409:
3407:
3404:
3402:
3399:
3397:
3394:
3392:
3389:
3387:
3384:
3382:
3379:
3377:
3374:
3372:
3369:
3367:
3364:
3363:
3361:
3358:
3353:
3347:
3344:
3342:
3339:
3338:
3336:
3333:
3328:
3322:
3319:
3317:
3314:
3312:
3309:
3308:
3306:
3303:
3298:
3292:
3289:
3288:
3286:
3283:
3278:
3272:
3269:
3267:
3264:
3262:
3259:
3257:
3254:
3252:
3249:
3247:
3244:
3242:
3239:
3238:
3236:
3233:
3228:
3225:
3223:
3219:
3209:
3206:
3202:
3199:
3197:
3194:
3193:
3192:
3189:
3187:
3184:
3183:
3180:
3177:
3173:
3163:
3160:
3159:
3156:
3150:
3147:
3146:
3144:
3141:
3136:
3130:
3127:
3125:
3122:
3120:
3117:
3116:
3114:
3111:
3110:
3104:
3098:
3095:
3093:
3090:
3088:
3085:
3084:
3082:
3079:
3078:
3072:
3066:
3063:
3061:
3058:
3056:
3053:
3051:
3048:
3046:
3043:
3041:
3038:
3036:
3033:
3032:
3030:
3027:
3026:
3020:
3017:
3015:
3011:
3001:
2998:
2996:
2993:
2991:
2988:
2986:
2983:
2981:
2978:
2977:
2975:
2972:
2967:
2961:
2958:
2956:
2953:
2951:
2948:
2947:
2945:
2942:
2941:
2935:
2929:
2926:
2924:
2921:
2920:
2918:
2915:
2914:
2908:
2902:
2899:
2898:
2896:
2893:
2892:
2886:
2880:
2877:
2875:
2872:
2870:
2867:
2865:
2862:
2861:
2859:
2856:
2855:
2849:
2846:
2844:
2840:
2834:
2831:
2829:
2826:
2825:
2823:
2821:
2817:
2807:
2804:
2803:
2801:
2798:
2795:
2789:
2783:
2780:
2779:
2777:
2774:
2772:
2766:
2760:
2757:
2755:
2752:
2751:
2749:
2746:
2745:
2739:
2736:
2734:
2730:
2725:
2724:Mitochondrial
2718:
2713:
2711:
2706:
2704:
2699:
2698:
2695:
2689:
2686:
2685:
2681:
2674:
2670:
2665:
2660:
2656:
2652:
2648:
2643:
2639:
2635:
2630:
2625:
2620:
2615:
2611:
2607:
2603:
2598:
2594:
2590:
2585:
2580:
2576:
2572:
2568:
2564:
2560:
2555:
2551:
2547:
2542:
2537:
2533:
2529:
2525:
2521:
2517:
2512:
2508:
2504:
2499:
2494:
2490:
2486:
2483:(6): 966–78.
2482:
2478:
2474:
2469:
2465:
2461:
2456:
2451:
2447:
2443:
2439:
2435:
2431:
2425:
2421:
2417:
2412:
2407:
2403:
2399:
2395:
2390:
2386:
2382:
2378:
2374:
2370:
2366:
2363:(3): 137–46.
2362:
2358:
2352:
2348:
2344:
2339:
2334:
2330:
2326:
2322:
2317:
2313:
2309:
2304:
2299:
2295:
2291:
2287:
2283:
2279:
2274:
2270:
2266:
2261:
2256:
2252:
2248:
2245:(7): 1600–6.
2244:
2240:
2236:
2231:
2227:
2223:
2218:
2213:
2208:
2203:
2199:
2195:
2191:
2187:
2183:
2178:
2174:
2170:
2165:
2160:
2156:
2152:
2148:
2143:
2139:
2135:
2130:
2125:
2121:
2117:
2114:(1): 187–92.
2113:
2109:
2105:
2100:
2096:
2092:
2087:
2082:
2078:
2074:
2070:
2066:
2062:
2057:
2053:
2049:
2044:
2039:
2034:
2029:
2025:
2021:
2017:
2012:
2008:
2004:
1999:
1994:
1990:
1986:
1982:
1978:
1974:
1969:
1965:
1961:
1957:
1953:
1949:
1945:
1941:
1937:
1933:
1929:
1924:
1920:
1916:
1911:
1906:
1901:
1896:
1892:
1888:
1884:
1880:
1876:
1871:
1867:
1863:
1859:
1855:
1852:(6): 339–45.
1851:
1847:
1842:
1838:
1834:
1830:
1826:
1822:
1818:
1814:
1810:
1804:
1800:
1796:
1791:
1786:
1782:
1778:
1774:
1770:
1766:
1761:
1757:
1753:
1749:
1745:
1741:
1737:
1731:
1730:
1725:
1710:
1706:
1700:
1697:
1692:
1688:
1682:
1679:
1674:
1670:
1665:
1660:
1656:
1652:
1648:
1644:
1640:
1633:
1630:
1625:
1621:
1616:
1611:
1606:
1601:
1597:
1593:
1589:
1582:
1579:
1574:
1570:
1565:
1560:
1556:
1552:
1548:
1541:
1538:
1527:
1523:
1517:
1514:
1509:
1505:
1499:
1496:
1491:
1487:
1482:
1477:
1473:
1469:
1465:
1461:
1457:
1449:
1446:
1441:
1437:
1432:
1427:
1422:
1417:
1413:
1409:
1408:BMC Neurology
1405:
1398:
1396:
1392:
1387:
1383:
1379:
1375:
1368:
1366:
1362:
1357:
1355:9780470547847
1351:
1347:
1340:
1338:
1336:
1332:
1327:
1321:
1319:
1315:
1310:
1306:
1300:
1297:
1292:
1288:
1282:
1279:
1275:
1271:
1266:
1264:
1262:
1258:
1254:
1250:
1245:
1243:
1241:
1237:
1231:
1229:
1227:
1223:
1215:
1213:
1211:
1207:
1203:
1199:
1195:
1191:
1187:
1183:
1179:
1176:, as well as
1175:
1171:
1167:
1163:
1159:
1155:
1151:
1147:
1143:
1139:
1135:
1131:
1127:
1119:
1117:
1115:
1107:
1103:
1095:
1091:
1087:
1083:
1079:
1075:
1071:
1063:
1061:
1059:
1055:
1050:
1046:
1038:
1036:
1033:
1030:domain and a
1029:
1028:transmembrane
1026:
1022:
1018:
1014:
1010:
1006:
1002:
998:
994:
990:
982:
977:
975:
973:
969:
965:
961:
957:
953:
949:
945:
941:
937:
930:
926:
921:
909:
904:
900:
896:
893:
889:
882:
880:
877:
873:
869:
865:
862:
858:
854:
847:
845:
839:
835:
832:
826:
820:
816:
813:
809:
802:
796:
792:
789:
783:
777:
773:
770:
768:RefSeq (mRNA)
766:
759:
758:
753:
749:
746:
740:
739:
734:
730:
727:
725:
721:
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631:
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531:mitochondrion
529:
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467:Gene ontology
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317:right auricle
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99:
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87:
83:
79:
75:
71:
67:
63:
59:
51:
46:
39:
34:
29:
25:
20:
3485:
3332:ATP synthase
3250:
3139:
3107:
3075:
3023:
2990:ATP synthase
2970:
2938:
2911:
2889:
2869:Cytochrome c
2852:
2792:
2782:Kynureninase
2769:
2742:
2654:
2650:
2609:
2605:
2569:(5): 883–6.
2566:
2562:
2523:
2519:
2480:
2476:
2437:
2433:
2404:(5): 842–6.
2401:
2397:
2360:
2356:
2328:
2324:
2288:(3): 671–6.
2285:
2281:
2242:
2238:
2192:(1): 171–6.
2189:
2185:
2154:
2150:
2111:
2107:
2068:
2064:
2023:
2020:BMC Genetics
2019:
1980:
1976:
1931:
1927:
1885:(2): 532–6.
1882:
1878:
1849:
1845:
1812:
1808:
1772:
1768:
1742:(2): 582–7.
1739:
1735:
1713:. Retrieved
1708:
1699:
1690:
1681:
1649:(5): 783–6.
1646:
1642:
1632:
1595:
1591:
1581:
1554:
1550:
1540:
1529:. Retrieved
1525:
1516:
1507:
1498:
1463:
1459:
1448:
1411:
1407:
1378:GeneReviews
1377:
1345:
1308:
1299:
1290:
1281:
1219:
1216:Interactions
1123:
1113:
1067:
1042:
986:
947:
935:
934:
928:
924:
841:
822:
798:
779:
755:
736:
710:
691:
665:
646:
626:
621:
375:hypothalamus
287:
280:
53:External IDs
3282:Complex III
1592:BMC Biology
1054:Archosauria
1049:RNA editing
1032:hydrophilic
1025:hydrophobic
521:respirasome
321:granulocyte
31:Identifiers
3502:Categories
3302:Complex IV
3109:urea cycle
2797:metabolism
2773:metabolism
2771:tryptophan
1715:2018-08-25
1711:. EMBL-EBI
1531:2017-02-20
1276:, May 2017
1255:, May 2017
1232:References
1182:spasticity
1082:ubiquinone
291:(ortholog)
74:HomoloGene
3486:see also
3232:Complex I
3040:Aconitase
1198:complex I
1178:hypotonia
1106:ubiquinol
1070:Complex I
1021:Complex I
978:Structure
970:(LS) and
966:(MELAS),
844:NP_904335
612:Orthologs
82:GeneCards
3513:Proteins
3186:Frataxin
3060:Fumarase
2726:proteins
2673:15814828
2638:15804362
2593:15791543
2550:15638829
2507:15467980
2464:15466285
2420:14764913
2377:14760490
2347:12949126
2312:12870132
2269:12840039
2226:12509511
2173:12436196
2138:12022039
2095:11938495
2052:11553319
2007:11349229
1964:52850476
1956:11130070
1866:16678300
1829:27742419
1799:24284231
1756:16023078
1673:22593086
1624:22839781
1573:12572620
1490:23965338
1440:24884847
1386:20301352
1272:–
1251:–
1064:Function
974:(LHON).
892:Wikidata
591:Sources:
526:membrane
403:duodenum
349:prostate
3346:MT-ATP8
3341:MT-ATP6
3261:MT-ND4L
2629:1079809
2584:1199377
2541:3905771
2498:1182158
2385:8413730
2303:1180693
2194:Bibcode
1998:1226134
1936:Bibcode
1919:7530363
1887:Bibcode
1837:6565853
1790:4035473
1664:3440978
1615:3473239
1481:4076475
1431:4047257
1414:: 116.
1274:Ensembl
1253:Ensembl
1130:MT-ATP6
1092:of the
1009:MT-ND4L
942:of the
724:UniProt
679:Ensembl
618:Species
597:QuickGO
266:pattern
168:10,404
155:10,059
38:Aliases
3451:MT-TS2
3446:MT-TS1
3416:MT-TL2
3411:MT-TL1
3321:MT-CO3
3316:MT-CO2
3311:MT-CO1
3291:MT-CYB
3271:MT-ND6
3266:MT-ND5
3256:MT-ND4
3251:MT-ND3
3246:MT-ND2
3241:MT-ND1
3014:Matrix
2671:
2636:
2626:
2612:: 26.
2591:
2581:
2548:
2538:
2505:
2495:
2462:
2455:524407
2452:
2418:
2383:
2375:
2345:
2310:
2300:
2267:
2260:403733
2257:
2224:
2217:140917
2214:
2171:
2136:
2129:384978
2126:
2093:
2086:447592
2083:
2050:
2040:
2026:: 13.
2005:
1995:
1962:
1954:
1928:Nature
1917:
1907:
1864:
1835:
1827:
1797:
1787:
1754:
1671:
1661:
1622:
1612:
1598:: 65.
1571:
1488:
1478:
1438:
1428:
1384:
1352:
1226:NDUFA9
1170:MT-CO3
1166:MT-ND6
1162:MT-ND5
1158:MT-ND4
1154:MT-ND2
1150:MT-ND1
1134:MT-TL1
1017:MT-ND6
1015:, and
1013:MT-ND5
1005:MT-ND4
1001:MT-ND2
997:MT-ND1
936:MT-ND3
929:MT-ND3
925:MT-ND3
878:search
876:PubMed
757:P03899
738:P03897
634:Entrez
437:BioGPS
246:9,806
233:9,459
70:102499
62:516002
3471:MT-TY
3466:MT-TW
3461:MT-TV
3456:MT-TT
3441:MT-TR
3436:MT-TQ
3431:MT-TP
3426:MT-TN
3421:MT-TM
3406:MT-TK
3401:MT-TI
3396:MT-TH
3391:MT-TG
3386:MT-TF
3381:MT-TE
3376:MT-TD
3371:MT-TC
3366:MT-TA
3162:PMPCB
3149:ALDH2
2971:other
2381:S2CID
2043:55343
1960:S2CID
1910:42775
1833:S2CID
1206:MELAS
1146:MT-TV
1142:MT-TW
1138:MT-TK
1108:(CoQH
938:is a
667:17718
627:Mouse
622:Human
593:Amigo
289:Mouse
282:Human
229:Start
187:Mouse
151:Start
107:Human
3357:tRNA
2669:PMID
2634:PMID
2589:PMID
2546:PMID
2503:PMID
2460:PMID
2416:PMID
2373:PMID
2343:PMID
2308:PMID
2265:PMID
2222:PMID
2169:PMID
2134:PMID
2091:PMID
2048:PMID
2003:PMID
1952:PMID
1915:PMID
1862:PMID
1825:PMID
1795:PMID
1752:PMID
1669:PMID
1620:PMID
1569:PMID
1486:PMID
1436:PMID
1382:PMID
1350:ISBN
1184:and
1168:and
1086:NADH
1074:NADH
940:gene
648:4537
272:Bgee
220:Band
204:Chr.
142:Band
124:Chr.
78:5018
58:OMIM
2659:doi
2624:PMC
2614:doi
2579:PMC
2571:doi
2536:PMC
2528:doi
2493:PMC
2485:doi
2450:PMC
2442:doi
2406:doi
2365:doi
2361:118
2333:doi
2298:PMC
2290:doi
2255:PMC
2247:doi
2212:PMC
2202:doi
2190:100
2159:doi
2124:PMC
2116:doi
2081:PMC
2073:doi
2038:PMC
2028:doi
1993:PMC
1985:doi
1944:doi
1932:408
1905:PMC
1895:doi
1854:doi
1817:doi
1785:PMC
1777:doi
1744:doi
1740:334
1659:PMC
1651:doi
1610:PMC
1600:doi
1559:doi
1476:PMC
1468:doi
1464:113
1426:PMC
1416:doi
1080:to
1047:or
824:n/a
800:n/a
781:n/a
445:n/a
387:lip
242:End
225:n/a
164:End
147:n/a
90:OMA
86:ND3
66:MGI
45:ND3
22:ND3
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
