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1、RESEARCH
2、REPORTSleadstoCdc48recruitmentforextractionand19.K.Kobayashietal.,Proc.Natl.Acad.Sci.U.S.A.107,forhelpprocessingRNA-seqdata;andD.Herschlaganddegradationoftheincompletetranslationproduct.17575–17579(2010).P.Harburyforhelpfulcomments.AminoacidanalysiswasRqc2p,throu
3、ghspecificbindingtoAla(IGC)and20.A.M.Burroughs,L.Aravind,RNABiol.11,360–372performedbyJ.ShulzeattheUniversityofCalifornia,Davis(2014).ProteomicsCore.EdmansequencingwasperformedatStanfordThr(IGU)tRNAs,directsthetemplate-freeand21.J.Chuetal.,Proc.Natl.Acad.Sci.U.S.A.106,209
4、7–2103University’sProteinandNucleicAcidFacilitybyD.Winant.This40S-freeelongationoftheincompletetransla-(2009).workwassupportedbytheSearleScholarsProgram(A.F.);tionproductwithCATtails.CATtailsinducea22.R.Ishimuraetal.,Science345,455–459(2014).StanfordUniversity(O.B.);NIHgr
5、ants1DP2GM110772-01(A.F.),heatshockresponsethroughamechanismthat23.S.D.Moore,R.T.Sauer,Annu.Rev.Biochem.76,101–124GM37949,andGM37951(A.M.L.);theCenterforRNASystems(2007).BiologygrantsP50GM102706(J.S.W.)andU01GM098254isyettobedetermined.24.A.Gerber,H.Grosjean,T.Melcher,W.K
6、eller,EMBOJ.17,(J.S.W.);andtheHowardHughesMedicalInstitute(J.S.W.).Hypomorphicmutationsinthemammalian4780–4789(1998).Theauthorsdeclarenocompetingfinancialinterests.ThehomologofLTN1causeneurodegenerationin25.K.W.Gastonetal.,NucleicAcidsRes.35,6740–6749cryo-EMstructureshave
7、beendepositedattheElectronmice(21).Similarly,micewithmutationsinacen-(2007).MicroscopyDataBank(accessioncodes2811,2812,6169,6170,Arg26.G.E.Crooks,G.Hon,J.M.Chandonia,S.E.Brenner,Genome6171,6172,6176,and6201).tralnervoussystem–specificisoformoftRNARes.14,1188–1190(2004).an
8、dGTPBP2,ahomologofyeastHbs1whichSUPPLEMENTARYMATERIALSworkswithPELOTA/Dom34todissociatestalledACKNOWLEDGMENTSwww.sciencemag.org/content/347/6217/75/suppl/DC180Sribosomes,sufferfromneurodegenerationElectronmicroscopywasperformedattheUniversityofUtahandMaterialsandMethodsth
9、eUniversityofCalifornia.WethankD.Belnap(Universityof(22).TheseobservationsrevealtheconsequencesF