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MT-TL1

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Houshmand M, Larsson NG, Oldfors A, Tulinius M, Holme E (March 1996). "Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A → G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne".
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Nishigaki Y, Tadesse S, Bonilla E, Shungu D, Hersh S, Keats BJ, Berlin CI, Goldberg MF, Vockley J, DiMauro S, Hirano M (May 2003). "A novel mitochondrial tRNA(Leu(UUR)) mutation in a patient with features of MERRF and Kearns-Sayre syndrome".
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We sequenced the mtDNA in the 11 probands, in their mothers and in 80 controls. We identified 33 diabetes-suspected mutations, 1/33 was 3243A>G. Most patients (91%) and their mothers had mutations in complex I and/or IV of the respiratory
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van den Bosch BJ, de Coo IF, Hendrickx AT, Busch HF, de Jong G, Scholte HR, Smeets HJ (October 2004). "Increased risk for cardiorespiratory failure associated with the A3302G mutation in the mitochondrial DNA encoded tRNALeu(UUR) gene".
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Anderson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG (April 1981). "Sequence and organization of the human mitochondrial genome".
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Kameoka K, Isotani H, Tanaka K, Azukari K, Fujimura Y, Shiota Y, Sasaki E, Majima M, Furukawa K, Haginomori S, Kitaoka H, Ohsawa N (April 1998). "Novel mitochondrial DNA mutation in tRNA(Lys) (8296A→G) associated with diabetes".
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Suzuki Y, Nishimaki K, Taniyama M, Muramatsu T, Atsumi Y, Matsuoka K, Ohta S (March 2004). "Lipoma and opthalmoplegia in mitochondrial diabetes associated with small heteroplasmy level of 3243 tRNA(Leu(UUR)) mutation".
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Ogle RF, Christodoulou J, Fagan E, Blok RB, Kirby DM, Seller KL, Dahl HH, Thorburn DR (January 1997). "Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin".
524:"Modification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes" 640:
Reardon W, Ross RJ, Sweeney MG, Luxon LM, Pembrey ME, Harding AE, Trembath RC (December 1992). "Diabetes mellitus associated with a pathogenic point mutation in mitochondrial DNA".
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Bulduk BK, Kiliç HB, Bekircan-Kurt CE, Haliloğlu G, Erdem Özdamar S, Topaloğlu H, Kocaefe YÇ (March 2020). "A Novel Amplification-Refractory Mutation System-PCR Strategy to Screen
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Iwanaga R, Koga Y, Aramaki S, Kato S, Kato H (February 2001). "Inter- and/or intra-organ distribution of mitochondrial C3303T or A3243G mutation in mitochondrial cytopathy".
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can result in multiple mitochondrial deficiencies and associated disorders. It is associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (
1201: 48: 1987: 1682: 893:"Mitochondrial tRNA(Leu(UUR)) mutation m.3302A > G presenting as childhood-onset severe myopathy: threshold determination through segregation study" 1677: 1768: 1350: 1436: 1245: 1194: 72: 2007: 1536: 1471: 1481: 1306: 1807: 1605: 1240: 60: 1974: 1802: 1466: 1187: 311: 53: 1797: 1583: 1541: 273: 269: 436: 1387: 1329: 1219: 314:, which may cause a wide variety of signs and symptoms affecting many organs and systems of the body, particularly the 1441: 848:
Yarham JW, Blakely EL, Alston CL, Roberts ME, Ealing J, Pal P, Turnbull DM, McFarland R, Taylor RW (February 2013).
1615: 1610: 1414: 1280: 850:"The m.3291T>C mt-tRNA(Leu(UUR)) mutation is definitely pathogenic and causes multisystem mitochondrial disease" 1340: 299:
gene were also described: m.3271 T > C, m.3291 T > C, m.3303 C > T, m.3256 C > T, and m.3260 A>G.
326:). These signs and symptoms can appear at any time from birth to adulthood. Phenotypes of the condition include 1531: 1409: 364: 1578: 1365: 891:
Ballhausen D, Guerry F, Hahn D, Schaller A, Nuoffer JM, Bonafé L, Jeannet PY, Jacquemont S (December 2010).
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molecule is a distinctive folded structure which contains three hairpin loops and resembles a three-leafed
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Blakely EL, de Silva R, King A, Schwarzer V, Harrower T, Dawidek G, Turnbull DM, Taylor RW (May 2005).
593:"Genetic, pathogenetic, and phenotypic implications of the mitochondrial A3243G tRNALeu(UUR) mutation" 1788: 1694: 1551: 1500: 1446: 696: 394: 347: 264:. A common mutation is A3243G. This mutation has been theorized to be associated with several other 454: 1718: 1486: 1360: 1167: 1129: 1092: 1019: 916: 808: 683:
Mazzaccara C, Iafusco D, Liguori R, Ferrigno M, Galderisi A, Vitale D, et al. (2012-04-19).
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gene pathogenic variants could be attributed to the alterations of mTERF binding efficiency.
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is a rare mitochondrial disorder known to affect many parts of the body, especially the
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GeneReviews/NCBI/NIH/UW entry on Mitochondrial DNA-Associated Leigh Syndrome and NARP
653: 609: 592: 578: 508: 253: 130: 1171: 1096: 920: 812: 669: 626: 1843: 1818: 1476: 1355: 1268: 1133: 1023: 422: 350:, and more. A 3302A>G mutation has been found in a patient with the deficiency. 281: 245: 126: 1045: 930:"LHON/MELAS overlap syndrome associated with a mitochondrial MTND1 gene mutation" 709: 89: 563: 184: 1595: 1257: 904: 865: 829: 796: 1526: 946: 929: 339: 237: 188: 1163: 1125: 1088: 1053: 955: 912: 883: 804: 760: 728: 618: 549: 540: 523: 500: 492: 179:(human mitochondrial map position 3230–3304) that transfers the amino acid 1117: 1015: 985: 768: 661: 414: 212:
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes
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Yasukawa T, Suzuki T, Ueda T, Ohta S, Watanabe K (February 2000).
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This article incorporates text from this source, which is in the
292:) and various problems with a patient's eyes, heart, or kidneys. 160: 136: 77: 1183: 685:"Mitochondrial diabetes in children: seek and you will find it" 176: 159:
at position 12 and it spans 75 base pairs. The structure of a
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mutations may result in complex I deficiency of the
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Biochemical and Biophysical Research Communications
100: 95: 83: 71: 59: 47: 39: 31: 26: 21: 479:Pathogenic Variants in Patient Repositories". 119:Mitochondrially encoded tRNA leucine 1 (UUA/G) 22:mitochondrially encoded tRNA leucine 1 (UUA/G) 1195: 236:. Symptoms of MELAS include recurrent severe 8: 1988:United States National Library of Medicine 1713: 1683:Mitochondrial permeability transition pore 1665: 1505: 1334: 1224: 1202: 1188: 1180: 1078: 945: 873: 718: 708: 608: 539: 1986:This article incorporates text from the 1678:Mitochondrial membrane transport protein 481:Genetic Testing and Molecular Biomarkers 16:Transfer RNA in the species Homo sapiens 1034:Diabetes Research and Clinical Practice 376: 1437:Cholesterol side-chain cleavage enzyme 897:Journal of Inherited Metabolic Disease 835:GeneReviews/NCBI/NIH/UW entry on MELAS 18: 322:, and the muscles used for movement ( 199:. Also, some studies showed that the 7: 854:Journal of the Neurological Sciences 470: 468: 1351:Coenzyme Q – cytochrome c reductase 528:The Journal of Biological Chemistry 260:, and other problems affecting the 1537:Oxoglutarate dehydrogenase complex 1472:Glycerol-3-phosphate dehydrogenase 1080:10.1111/j.1600-0404.1999.tb00670.x 934:European Journal of Human Genetics 129:which in humans is encoded by the 14: 1482:Carnitine palmitoyltransferase II 1606:Carbamoyl phosphate synthetase I 1246:Long-chain-fatty-acid—CoA ligase 1241:Carnitine palmitoyltransferase I 610:10.1111/j.1600-0404.2007.00836.x 572: 175:MT-TL1 is a small 75 nucleotide 1467:Glutamate aspartate transporter 312:mitochondrial respiratory chain 1584:Pyruvate dehydrogenase complex 1542:Succinyl coenzyme A synthetase 274:Diabetes mellitus and deafness 270:diabetes mellitus and deafness 252:-like episodes with a loss of 1: 1156:10.1016/S0960-8966(02)00283-3 1067:Acta Neurologica Scandinavica 1046:10.1016/j.diabres.2003.10.024 978:10.1016/S0022-3476(97)70323-8 597:Acta Neurologica Scandinavica 295:Other rare point variants on 1388:Dihydroorotate dehydrogenase 710:10.1371/journal.pone.0034956 654:10.1016/0140-6736(92)92560-3 1442:Steroid 11-beta-hydroxylase 2024: 1616:N-Acetylglutamate synthase 1611:Ornithine transcarbamylase 1415:Glycerol phosphate shuttle 1281:monoamine neurotransmitter 2008:Human mitochondrial genes 1970: 1668: 1644: 1341:oxidative phosphorylation 966:The Journal of Pediatrics 905:10.1007/s10545-010-9098-2 866:10.1016/j.jns.2012.12.003 797:10.1016/j.nmd.2004.06.004 591:Finsterer J (July 2007). 459:Learn Science at Scitable 1532:Isocitrate dehydrogenase 1410:Malate-aspartate shuttle 365:Mitochondrial Myopathies 1579:Glutamate dehydrogenase 1366:Succinate dehydrogenase 1144:Neuromuscular Disorders 947:10.1038/sj.ejhg.5201363 899:. 33 Suppl 3: S219-26. 785:Neuromuscular Disorders 568:Genetics Home Reference 151:gene is located on the 1975:mitochondrial diseases 1574:Aspartate transaminase 1231:fatty acid degradation 761:10.1006/bbrc.1998.8437 541:10.1074/jbc.275.6.4251 493:10.1089/gtmb.2019.0079 266:mitochondrial diseases 1688:Mitochondrial carrier 1564:anaplerotic reactions 1400:mitochondrial shuttle 1378:pyrimidine metabolism 1118:10.1007/s004010000266 1106:Acta Neuropathologica 455:"tRNA / transfer RNA" 207:Clinical significance 1695:Translocator protein 1552:Malate dehydrogenase 1447:Aldosterone synthase 441:www.ncbi.nlm.nih.gov 348:exercise intolerance 303:Complex I deficiency 276:is characterized by 1307:Intermembrane space 701:2012PLoSO...734956M 399:1981Natur.290..457A 240:, muscle weakness ( 1990:, which is in the 1662:Other/to be sorted 1627:alcohol metabolism 1487:Uncoupling protein 1361:NADH dehydrogenase 1008:10.1007/BF02185750 1982: 1981: 1966: 1965: 1709:Mitochondrial DNA 1703: 1702: 1657: 1656: 1512:citric acid cycle 1495: 1494: 1301: 1300: 1293:Monoamine oxidase 360:Mitochondrial DNA 195:synthesis during 157:mitochondrial DNA 116: 115: 2015: 1846: 1821: 1791: 1771: 1721: 1714: 1666: 1629: 1599: 1567: 1522:Citrate synthase 1515: 1506: 1460: 1430: 1403: 1381: 1344: 1335: 1315:Adenylate kinase 1286: 1262: 1234: 1225: 1204: 1197: 1190: 1181: 1175: 1137: 1100: 1082: 1057: 1027: 989: 959: 949: 924: 887: 877: 817: 816: 779: 773: 772: 743: 737: 736: 722: 712: 680: 674: 673: 648:(8832): 1376–9. 637: 631: 630: 612: 588: 582: 576: 575: 571: 560: 554: 553: 543: 519: 513: 512: 472: 463: 462: 451: 445: 444: 433: 427: 426: 407:10.1038/290457a0 393:(5806): 457–65. 381: 324:skeletal muscles 19: 2023: 2022: 2018: 2017: 2016: 2014: 2013: 2012: 1998: 1997: 1983: 1978: 1962: 1842: 1837: 1817: 1812: 1787: 1782: 1767: 1762: 1717: 1699: 1653: 1640: 1625: 1620: 1593: 1588: 1561: 1556: 1509: 1491: 1456: 1451: 1427:steroidogenesis 1424: 1419: 1397: 1392: 1375: 1370: 1338: 1324: 1320:Creatine kinase 1297: 1283: 1278: 1273: 1255: 1250: 1228: 1214: 1208: 1178: 1140: 1103: 1060: 1030: 992: 962: 927: 890: 847: 843: 841:Further reading 826: 821: 820: 781: 780: 776: 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287: 283: 279: 275: 271: 267: 263: 259: 255: 254:consciousness 251: 247: 243: 239: 235: 231: 227: 223: 219: 216:Mutations in 211: 206: 204: 202: 198: 194: 190: 187:chain at the 186: 183:to a growing 182: 178: 170: 168: 166: 162: 158: 154: 150: 142: 140: 138: 135: 132: 131:mitochondrial 128: 124: 120: 112: 111: 108: 105: 103: 99: 94: 91: 88: 86: 82: 79: 76: 74: 70: 67: 64: 62: 58: 55: 52: 50: 46: 42: 38: 34: 30: 25: 20: 1985: 1984: 1972: 1897: 1819:ATP synthase 1626: 1594: 1562: 1510: 1477:ATP synthase 1457: 1425: 1398: 1376: 1356:Cytochrome c 1339: 1279: 1269:Kynureninase 1256: 1229: 1147: 1143: 1109: 1105: 1073:(2): 125–9. 1070: 1066: 1040:(3): 225–9. 1037: 1033: 999: 995: 969: 965: 940:(5): 623–7. 937: 933: 896: 857: 853: 788: 784: 777: 755:(2): 523–7. 752: 748: 741: 732: 692: 688: 678: 645: 641: 635: 600: 596: 586: 567: 564:"MT-TH gene" 558: 531: 527: 517: 484: 480: 476: 458: 449: 440: 431: 390: 386: 379: 307: 306: 296: 294: 282:hearing loss 268:, including 246:hearing loss 217: 215: 200: 174: 148: 146: 133: 127:transfer RNA 122: 118: 117: 109: 40:Alt. symbols 1769:Complex III 603:(1): 1–14. 197:translation 185:polypeptide 27:Identifiers 1789:Complex IV 1596:urea cycle 1284:metabolism 1260:metabolism 1258:tryptophan 371:References 96:Other data 1973:see also 1719:Complex I 1527:Aconitase 509:212693790 340:hypotonia 238:headaches 143:Structure 90:NC_001807 49:NCBI gene 2002:Category 1673:Frataxin 1547:Fumarase 1213:proteins 1172:53265571 1164:12868503 1126:11271374 1097:12099444 1089:10071173 1054:14757294 956:15657614 921:24006011 913:20458543 884:23273904 813:38721676 805:15351426 729:22536343 689:PLOS ONE 670:34540249 627:43823627 619:17587249 550:10660592 501:32167396 354:See also 336:dystonia 332:epilepsy 290:myopathy 278:diabetes 258:seizures 242:myopathy 232:and the 191:site of 189:ribosome 171:Function 1833:MT-ATP8 1828:MT-ATP6 1748:MT-ND4L 1134:1970758 1024:6688307 1016:8786060 986:9003864 875:3560033 769:9571188 720:3334935 697:Bibcode 662:1360090 423:4355527 415:7219534 395:Bibcode 344:myalgia 193:protein 181:leucine 155:of the 107:Chr. MT 1938:MT-TS2 1933:MT-TS1 1903:MT-TL2 1898:MT-TL1 1808:MT-CO3 1803:MT-CO2 1798:MT-CO1 1778:MT-CYB 1758:MT-ND6 1753:MT-ND5 1743:MT-ND4 1738:MT-ND3 1733:MT-ND2 1728:MT-ND1 1501:Matrix 1170:  1162:  1132:  1124:  1095:  1087:  1052:  1022:  1014:  984:  954:  919:  911:  882:  872:  811:  803:  767:  734:chain. 727:  717:  668:  660:  642:Lancet 625:  617:  548:  507:  499:  477:MT-TL1 421:  413:  387:Nature 318:, the 297:MT-TL1 250:stroke 218:MT-TL1 201:MT-TL1 165:clover 149:MT-TL1 134:MT-TL1 123:MT-TL1 85:RefSeq 78:590050 35:MT-TL1 32:Symbol 1958:MT-TY 1953:MT-TW 1948:MT-TV 1943:MT-TT 1928:MT-TR 1923:MT-TQ 1918:MT-TP 1913:MT-TN 1908:MT-TM 1893:MT-TK 1888:MT-TI 1883:MT-TH 1878:MT-TG 1873:MT-TF 1868:MT-TE 1863:MT-TD 1858:MT-TC 1853:MT-TA 1649:PMPCB 1636:ALDH2 1458:other 1168:S2CID 1130:S2CID 1093:S2CID 1020:S2CID 917:S2CID 809:S2CID 666:S2CID 623:S2CID 505:S2CID 419:S2CID 320:heart 308:MT-TP 234:brain 226:MELAS 222:MELAS 153:p arm 125:is a 102:Locus 43:MTTL1 1844:tRNA 1160:PMID 1122:PMID 1085:PMID 1050:PMID 1012:PMID 982:PMID 952:PMID 909:PMID 880:PMID 801:PMID 765:PMID 725:PMID 658:PMID 615:PMID 546:PMID 497:PMID 411:PMID 161:tRNA 147:The 137:gene 73:OMIM 66:7490 61:HGNC 54:4567 1152:doi 1114:doi 1110:101 1075:doi 1042:doi 1004:doi 974:doi 970:130 942:doi 901:doi 870:PMC 862:doi 858:325 793:doi 757:doi 753:245 715:PMC 705:doi 650:doi 646:340 605:doi 601:116 536:doi 532:275 489:doi 403:doi 391:290 244:), 224:). 177:RNA 2004:: 1166:. 1158:. 1148:13 1146:. 1128:. 1120:. 1108:. 1091:. 1083:. 1071:99 1069:. 1065:. 1048:. 1038:63 1036:. 1018:. 1010:. 1000:97 998:. 980:. 968:. 950:. 938:13 936:. 932:. 915:. 907:. 895:. 878:. 868:. 856:. 852:. 807:. 799:. 789:14 787:. 763:. 751:. 731:. 723:. 713:. 703:. 691:. 687:. 664:. 656:. 644:. 621:. 613:. 599:. 595:. 566:. 544:. 530:. 526:. 503:. 495:. 485:24 483:. 467:^ 457:. 439:. 417:. 409:. 401:. 389:. 346:, 342:, 338:, 334:, 330:, 272:. 256:, 248:, 167:. 139:. 1994:. 1203:e 1196:t 1189:v 1174:. 1154:: 1136:. 1116:: 1099:. 1077:: 1056:. 1044:: 1026:. 1006:: 988:. 976:: 958:. 944:: 923:. 903:: 886:. 864:: 815:. 795:: 771:. 759:: 707:: 699:: 693:7 672:. 652:: 629:. 607:: 581:. 570:. 552:. 538:: 511:. 491:: 461:. 443:. 425:. 405:: 397:: 288:(

Index

NCBI gene
4567
HGNC
7490
OMIM
590050
RefSeq
NC_001807
Locus
Chr. MT

transfer RNA
mitochondrial
gene
p arm
mitochondrial DNA
tRNA
clover
RNA
leucine
polypeptide
ribosome
protein
translation
MELAS
MELAS
nervous system
brain
headaches
myopathy

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