{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T03:21:45Z","timestamp":1789356105271,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"1-2","license":[{"start":{"date-parts":[[2004,11,16]],"date-time":"2004-11-16T00:00:00Z","timestamp":1100563200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FEBS Letters"],"published-print":{"date-parts":[[2004,12,3]]},"abstract":"<jats:p>The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke\u2010like episodes syndrome (MELAS) is a rare congenital disorder of mitochondrial DNA. Five single nucleotide substitutions within the human mitochondrial tRNA<jats:sup>Leu<\/jats:sup>(UUR) gene have been reported to be associated with MELAS. Here, we provide in vitro evidence that the aminoacylation capacities of these five hmtRNA<jats:sup>Leu<\/jats:sup>(UUR) transcripts are reduced to different extents relative to the wild\u2010type hmtRNA<jats:sup>Leu<\/jats:sup>(UUR) transcript. A thermal denaturation experiment showed that the A3243G and T3291C mutants, which were the least charged by LeuRS, have fragile structures. In addition, the T3291C mutant can inhibit aminoacylation of the wild\u2010type hmtRNA<jats:sup>Leu<\/jats:sup>(UUR), indicating that it may act as an inhibitor in the mitochondrial heteroplasmic environment.<\/jats:p>","DOI":"10.1016\/j.febslet.2004.11.004","type":"journal-article","created":{"date-parts":[[2004,11,18]],"date-time":"2004-11-18T06:37:05Z","timestamp":1100759825000},"page":"135-139","source":"Crossref","is-referenced-by-count":25,"title":["Reduction of mitochondrial tRNA<sup>Leu<\/sup>(UUR) aminoacylation by some MELAS\u2010associated mutations"],"prefix":"10.1002","volume":"578","author":[{"given":"Rui","family":"Hao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong-Neng","family":"Yao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong-Gang","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min-Gang","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"En-Duo","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,11,16]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01936918"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1355838200001047"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2003.09.006"},{"key":"e_1_2_6_5_1","first-page":"351","article-title":"MELAS as an example of a mitochondrial disease","volume":"42","author":"Piechota J.","year":"2001","journal-title":"J. Appl. Genet."},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/348651a0"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.12.2081"},{"key":"e_1_2_6_8_1","first-page":"1055","article-title":"A mitochondrial tRNALeu(UUR) mutation at 3,256 associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)","volume":"33","author":"Sato W.","year":"1994","journal-title":"Biochem. Mol. Biol. Int."},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(91)90042-8"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1994.2119"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M908734199"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi026882r"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(03)00373-5"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(02)03833-4"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1046-5928(03)00097-4"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.275.15.11207"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.16.11199"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1006\/prep.1999.1083"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(98)01096-5"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(89)80108-9"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.33.22968"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2004.02.008"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/26.7.1636"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2004.03.066"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/79612"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M008320200"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1261\/rna.5267604"},{"key":"e_1_2_6_28_1","first-page":"586","article-title":"Dimerization of a pathogenic human mitochondrial tRNA","volume":"9","author":"Wittenhagen L.M.","year":"2002","journal-title":"Nat. Struct. Biol."},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.embor.embor713"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.790568"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00115-7"}],"container-title":["FEBS Letters"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1016%2Fj.febslet.2004.11.004","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.febslet.2004.11.004","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T07:59:55Z","timestamp":1697097595000},"score":1,"resource":{"primary":{"URL":"https:\/\/febs.onlinelibrary.wiley.com\/doi\/10.1016\/j.febslet.2004.11.004"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,11,16]]},"references-count":30,"journal-issue":{"issue":"1-2","published-print":{"date-parts":[[2004,12,3]]}},"alternative-id":["10.1016\/j.febslet.2004.11.004"],"URL":"https:\/\/doi.org\/10.1016\/j.febslet.2004.11.004","archive":["Portico"],"relation":{},"ISSN":["0014-5793","1873-3468"],"issn-type":[{"value":"0014-5793","type":"print"},{"value":"1873-3468","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,11,16]]}}}