{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T04:00:42Z","timestamp":1793678442104,"version":"4.5.1"},"reference-count":68,"publisher":"National Academy of Sciences","issue":"32","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2014,8,12]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>Cellular RNAs undergo assembly with various proteins, which leads to the formation of functional ribonucleoprotein (RNP) complexes. Kinetic defects in the RNP assembly pathway, which affect the rate of RNP formation, can lead to a reduction in the levels of functional RNPs in the cell, and can lead to a disease state, which we classify as an \u201cRNP assembly disease.\u201d One example of this class of diseases is spinal muscular atrophy (SMA), where mutations in the assembly factor survival motor neuron lead to reduced small nuclear (sn)RNA and snRNP levels. Here we describe the decay pathways that regulate snRNA levels and function in the cell, prevention of which could be pertinent as a therapy for SMA.<\/jats:p>","DOI":"10.1073\/pnas.1412614111","type":"journal-article","created":{"date-parts":[[2014,7,29]],"date-time":"2014-07-29T02:39:50Z","timestamp":1406601590000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":54,"title":["Quality control of assembly-defective U1 snRNAs by decapping and 5\u2032-to-3\u2032 exonucleolytic digestion"],"prefix":"10.1073","volume":"111","author":[{"given":"Siddharth","family":"Shukla","sequence":"first","affiliation":[{"name":"Department of Chemistry and Biochemistry and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roy","family":"Parker","sequence":"additional","affiliation":[{"name":"Department of Chemistry and Biochemistry and"},{"name":"Howard Hughes Medical Institute, University of Colorado, Boulder, CO 80303"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2014,7,28]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2009.01.019"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2007.10.041"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1990.tb07436.x"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/43694"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1990.tb08231.x"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq147"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2008.03.009"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1101\/sqb.2006.71.001"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/wrna.87"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.25.13.5543-5551.2005"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.03.031"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp157"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2012.09.012"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0000921"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1319280110"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrn2670"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1000773"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2012.05.014"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1261\/rna.038919.113"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.342005"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt567"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.4.10.1766"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/27.2.587"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1534\/genetics.111.137265"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2008.07.003"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2005.04.030"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1183804"},{"key":"e_1_3_3_29_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.0030189"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2005.04.029"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdf448"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb.2381"},{"key":"e_1_3_3_33_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature09338"},{"key":"e_1_3_3_34_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2013.05.012"},{"key":"e_1_3_3_35_2","doi-asserted-by":"publisher","DOI":"10.1093\/genetics\/157.1.27"},{"key":"e_1_3_3_36_2","doi-asserted-by":"publisher","DOI":"10.1002\/yea.1015"},{"key":"e_1_3_3_37_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.21.8.2815-2825.2001"},{"key":"e_1_3_3_38_2","doi-asserted-by":"publisher","DOI":"10.1002\/wrna.15"},{"key":"e_1_3_3_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2010.10.010"},{"key":"e_1_3_3_40_2","doi-asserted-by":"publisher","DOI":"10.1261\/rna.2439811"},{"key":"e_1_3_3_41_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.str.2005.11.010"},{"key":"e_1_3_3_42_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1074962"},{"key":"e_1_3_3_43_2","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1101-945"},{"key":"e_1_3_3_44_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0508947102"},{"key":"e_1_3_3_45_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkt1271"},{"key":"e_1_3_3_46_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1082320"},{"key":"e_1_3_3_47_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2007.02.011"},{"key":"e_1_3_3_48_2","doi-asserted-by":"publisher","DOI":"10.1261\/rna.037309.112"},{"key":"e_1_3_3_49_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.01727-07"},{"key":"e_1_3_3_50_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.25.22.9764-9772.2005"},{"key":"e_1_3_3_51_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1120090109"},{"key":"e_1_3_3_52_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2013.02.023"},{"key":"e_1_3_3_53_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq508"},{"key":"e_1_3_3_54_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(86)90072-3"},{"key":"e_1_3_3_55_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt258"},{"key":"e_1_3_3_56_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt257"},{"key":"e_1_3_3_57_2","doi-asserted-by":"publisher","DOI":"10.1021\/cb800120t"},{"key":"e_1_3_3_58_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00205-7"},{"key":"e_1_3_3_59_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32053"},{"key":"e_1_3_3_60_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31331"},{"key":"e_1_3_3_61_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt204"},{"key":"e_1_3_3_62_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergen.2011.11.002"},{"key":"e_1_3_3_63_2","doi-asserted-by":"publisher","DOI":"10.1038\/35096585"},{"key":"e_1_3_3_64_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1476206"},{"key":"e_1_3_3_65_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1200587"},{"key":"e_1_3_3_66_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1202205"},{"key":"e_1_3_3_67_2","doi-asserted-by":"publisher","DOI":"10.1261\/rna.045187.114"},{"key":"e_1_3_3_68_2","doi-asserted-by":"publisher","DOI":"10.1017\/S1355838299990829"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.1412614111","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,12]],"date-time":"2022-04-12T23:37:22Z","timestamp":1649806642000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.1412614111"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,7,28]]},"references-count":68,"journal-issue":{"issue":"32","published-print":{"date-parts":[[2014,8,12]]}},"alternative-id":["10.1073\/pnas.1412614111"],"URL":"https:\/\/doi.org\/10.1073\/pnas.1412614111","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,7,28]]},"assertion":[{"value":"2014-07-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}