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dc.contributor.authorJourdain, Alexis A.-
dc.contributor.authorSimarro-Grande, María-
dc.contributor.authorMartinou, Jean-Claude-
dc.date.accessioned2017-11-23T12:55:30Z-
dc.date.available2017-11-23T12:55:30Z-
dc.date.issued2015-
dc.identifierdoi: 10.1016/j.celrep.2015.01.063-
dc.identifierissn: 2211-1247-
dc.identifier.citationCell Reports 10(7): 1110-1121 (2015)-
dc.identifier.urihttp://hdl.handle.net/10261/157664-
dc.descriptionUnder a Creative Commons license.-- et al.-
dc.description.abstractThe mitochondrial genome relies heavily on post-transcriptional 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.sponsorshipThis 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.publisherElsevier-
dc.relation.isversionofPublisher's version-
dc.rightsopenAccess-
dc.titleA mitochondria-specific isoform of FASTK is present in mitochondrial RNA granules and regulates gene expression and function-
dc.typeartículo-
dc.identifier.doi10.1016/j.celrep.2015.01.063-
dc.relation.publisherversionhttps://doi.org/10.1016/j.celrep.2015.01.063-
dc.date.updated2017-11-23T12:55:30Z-
dc.description.versionPeer Reviewed-
dc.language.rfc3066eng-
dc.rights.licensehttp://creativecommons.org/licenses/by-nc-nd/3.0/-
dc.contributor.funderRoche-
dc.contributor.funderJunta de Castilla y León-
dc.contributor.funderSwiss National Science Foundation-
dc.relation.csicNo-
dc.identifier.funderhttp://dx.doi.org/10.13039/100004337es_ES
dc.identifier.funderhttp://dx.doi.org/10.13039/501100014180es_ES
dc.type.coarhttp://purl.org/coar/resource_type/c_6501es_ES
item.cerifentitytypePublications-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.grantfulltextopen-
item.openairetypeartículo-
item.fulltextWith Fulltext-
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Este item está licenciado bajo una Licencia Creative Commons Creative Commons