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The efficiency of various classes of compounds to suppress PTCs in mammalian cells is discussed along with the current limitations of this therapy. We also elaborate on approaches to improve the efficiency of suppression that include methods to enhance the effectiveness of current suppression drugs and the design or discovery of new, more effective suppression agents. Finally, we discuss the role of nonsense\u2010mediated mRNA decay (NMD) in limiting the effectiveness of suppression therapy, and describe tactics that may allow the efficiency of NMD to be modulated in order to enhance suppression therapy. <jats:italic>WIREs RNA<\/jats:italic> 2011 2 837\u2013852 DOI: 10.1002\/wrna.95<\/jats:p><jats:p>Correction added on July 13 2011, after first online publication. Figure 5 was incorrect and has been replaced.<\/jats:p><jats:p>This article is categorized under:\n<jats:list list-type=\"explicit-label\">\n<jats:list-item><jats:p>Translation &gt; Translation Mechanisms<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>RNA in Disease and Development &gt; RNA in Disease<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p>","DOI":"10.1002\/wrna.95","type":"journal-article","created":{"date-parts":[[2011,7,6]],"date-time":"2011-07-06T13:31:29Z","timestamp":1309959089000},"page":"837-852","source":"Crossref","is-referenced-by-count":90,"title":["Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases"],"prefix":"10.1002","volume":"2","author":[{"given":"Kim M.","family":"Keeling","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Bedwell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,7,6]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.10.1893"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2004.11.048"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1995.tb00078.x"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80667-4"},{"key":"e_1_2_7_6_2","first-page":"334","article-title":"Eukaryotic polypeptide chain release factor eRF3 is an eRF1\u2010 and ribosome\u2010dependent guanosine triphosphatase.","volume":"2","author":"Frolova L","year":"1996","journal-title":"RNA"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.24.17.7769-7778.2004"},{"key":"e_1_2_7_8_2","doi-asserted-by":"crossref","first-page":"17802","DOI":"10.1016\/S0021-9258(17)32379-7","article-title":"Premature translation termination mutations are efficiently suppressed in a highly conserved region of yeast Ste6p, a member of the ATP\u2010binding cassette (ABC) transporter family.","volume":"269","author":"Fearon K","year":"1994","journal-title":"J Biol Chem"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2199-2-3"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1017\/S1355838200000716"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.12.5431"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1139\/o95-118"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.3.291"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1023474719042"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-001-0317-z"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature03060"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.24.16677"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1038\/emboj.2008.17"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.0060111"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1101\/sqb.2001.66.217"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.1093\/embo-reports\/kve176"},{"key":"e_1_2_7_22_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/27.1.293"},{"key":"e_1_2_7_23_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1067338"},{"key":"e_1_2_7_24_2","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb1256"},{"key":"e_1_2_7_25_2","doi-asserted-by":"publisher","DOI":"10.2174\/1568026033452131"},{"key":"e_1_2_7_26_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/18.11.3133"},{"key":"e_1_2_7_27_2","doi-asserted-by":"publisher","DOI":"10.1006\/jmbi.2000.4420"},{"key":"e_1_2_7_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2005.03.025"},{"key":"e_1_2_7_29_2","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1056\/NEJMra0907180","article-title":"New therapeutic approaches to mendelian disorders.","volume":"363","author":"Dietz HC.","year":"2011","journal-title":"N Engl J Med"},{"key":"e_1_2_7_30_2","volume-title":"Recoding: Expansion of Decoding Rules Enriches Gene Expression.","author":"Keeling KM","year":"2010"},{"key":"e_1_2_7_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2008.08.010"},{"key":"e_1_2_7_32_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.163.7.2004001"},{"key":"e_1_2_7_33_2","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2006-0173OC"},{"key":"e_1_2_7_34_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.161.3.9904116"},{"key":"e_1_2_7_35_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa022170"},{"key":"e_1_2_7_36_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10341"},{"key":"e_1_2_7_37_2","first-page":"15","article-title":"Gentamicin administration in Duchenne patients with premature stop codon. 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