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dc.contributor.authorJourdain, Alexis A.
dc.contributor.authorKoppen, Mirko
dc.contributor.authorRodley, Christopher D.
dc.contributor.authorMaundrell, Kinsey
dc.contributor.authorGueguen, Naig
dc.contributor.authorReynier, Pascal
dc.contributor.authorGuaras, Adela 
dc.contributor.authorEnriquez, José Antonio 
dc.contributor.authorAnderson, Paul
dc.contributor.authorSimarro, Maria
dc.contributor.authorMartinou, Jean-Claude
dc.date.accessioned2017-12-01T07:37:25Z
dc.date.available2017-12-01T07:37:25Z
dc.date.issued2015
dc.identifierISI:000349918700008
dc.identifier.issn2211-1247
dc.identifier.urihttp://hdl.handle.net/20.500.12105/5513
dc.description.abstractThe mitochondrial genome relies heavily on posttranscriptional events for its proper expression, and misregulation of this process can cause mitochondrial genetic diseases in humans. Here, we report that a novel translational variant of Fas-activated serine/threonine kinase (FASTK) co-localizes with mitochondrial RNA granules and is required for the biogenesis of ND6 mRNA, a mitochondrial-encoded subunit of the NADH dehydrogenase complex (complex I). We show that ablating FASTK expression in cultured cells and mice results specifically in loss of ND6 mRNA and reduced complex I activity in vivo. FASTK binds at multiple sites along the ND6 mRNA and its precursors and cooperates with the mitochondrial degradosome to ensure regulated ND6 mRNA biogenesis. These data provide insights into the mechanism and control of mitochondrial RNA processing within mitochondrial RNA granules.
dc.description.sponsorshipWe would like to thank Y. Brixner and S. Montessuit for their technical support, all members of the J.-C.M. lab for their comments on the manuscript, and M. Zeviani, C. Viscomi, Z. Chrzanowska-Lightowlers, R.N. Lightowlers, M. Goldschmidt-Clermont, S. Thore, D. Martinvalet, G. Voeltz, D. Picard, F. Stutz, and their laboratories for sharing reagents and for intellectual input during the project. This work was supported by the Swiss National Science Foundation (31993A-141068/1), IGE3, and the State of Geneva. MS work was supported by the Gerencia Regional de Salud de la JCyL (GRS 642/A/11) and Roche Diagnostics.
dc.language.isoeng
dc.publisherCELL PRESS
dc.relation.isversionofPublisher's version
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectNADH-DEHYDROGENASE
dc.subjectBINDING PROTEINS
dc.subjectMESSENGER-RNAS
dc.subjectRIBOSOMAL-RNA
dc.subjectND6 SUBUNIT
dc.subjectCOMPLEX
dc.subjectTRANSLATION
dc.subjectDNA
dc.subjectPOLYADENYLATION
dc.subjectTRANSCRIPTOME
dc.titleA Mitochondria-Specific Isoform of FASTK Is Present In Mitochondrial RNA Granules and Regulates Gene Expression and Function
dc.typeArtículo
dc.rights.licenseAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.identifier.pubmedID25704814
dc.format.volume10
dc.format.page1110-1121
dc.identifier.doi10.1016/j.celrep.2015.01.063
dc.contributor.funderSwiss National Science Foundation
dc.contributor.funderJunta de Castilla y León
dc.contributor.funderRoche
dc.description.peerreviewed
dc.relation.publisherversionhttps://doi.org/10.1016/j.celrep.2015.01.063
dc.identifier.journalCell Reports
dc.identifier.journalCell Rep. 2015; 10(7):1110-21
dc.repisalud.orgCNICCNIC::Grupos de investigación::Genética Funcional del Sistema de Fosforilación Oxidativa
dc.repisalud.institucionCNIC
dc.rights.accessRightsinfo:eu-repo/semantics/openAccesses_ES


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Attribution-NonCommercial-NoDerivatives 4.0 Internacional
This item is licensed under a: Attribution-NonCommercial-NoDerivatives 4.0 Internacional