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The overall structures of wildtype and S581L\u2010GlyRS, including the active site, are very similar. However, residues 567\u2013575 of the anticodon\u2010binding domain shift position and in turn could indirectly affect glycine binding via the tRNA or alternatively inhibit conformational changes. Reduced enzyme activity may underlie neuronal degeneration, although a dominant\u2010negative effect is more likely in this autosomal dominant disorder.<\/jats:p>","DOI":"10.1016\/j.febslet.2007.05.046","type":"journal-article","created":{"date-parts":[[2007,5,30]],"date-time":"2007-05-30T11:58:01Z","timestamp":1180526281000},"page":"2959-2964","source":"Crossref","is-referenced-by-count":35,"title":["Crystal structure of human wildtype and S581L\u2010mutant glycyl\u2010tRNA synthetase, an enzyme underlying distal spinal muscular atrophy"],"prefix":"10.1002","volume":"581","author":[{"given":"Muhammed Z.","family":"Cader","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingshan","family":"Ren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul A.","family":"James","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise E.","family":"Bird","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Talbot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David K.","family":"Stammers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,5,29]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1327.1998.2510744.x"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2005.01.045"},{"key":"e_1_2_6_4_1","first-page":"954","article-title":"Aminoacyl-tRNA synthetases in biology and disease: new evidence for structural and functional diversity in an ancient family of enzymes","volume":"3","author":"Francklyn C.","year":"1997","journal-title":"RNA"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/5.9.1373"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1086\/375039"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(98)00264-0"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.9.1629"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s100480050013"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh590"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000218304.02715.04"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000201275.18875.ac"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000242619.52335.bc"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1727"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature05096"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1107\/S0907444905007808"},{"key":"e_1_2_6_17_1","doi-asserted-by":"crossref","unstructured":"Collaborative Computational Project Number 4 (1994) The CCP4 suite: programs for protein crystallography Acta Crystallogr. 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