Mostrar el registro sencillo del ítem
dc.contributor.author | Jourdain, Alexis A. | |
dc.contributor.author | Koppen, Mirko | |
dc.contributor.author | Rodley, Christopher D. | |
dc.contributor.author | Maundrell, Kinsey | |
dc.contributor.author | Gueguen, Naig | |
dc.contributor.author | Reynier, Pascal | |
dc.contributor.author | Guaras, Adela | |
dc.contributor.author | Enriquez, Jose Antonio | |
dc.contributor.author | Anderson, Paul | |
dc.contributor.author | Simarro, Maria | |
dc.contributor.author | Martinou, Jean-Claude | |
dc.date.accessioned | 2017-12-01T07:37:25Z | |
dc.date.available | 2017-12-01T07:37:25Z | |
dc.date.issued | 2015 | |
dc.identifier | ISI:000349918700008 | |
dc.identifier.issn | 2211-1247 | |
dc.identifier.uri | http://hdl.handle.net/20.500.12105/5513 | |
dc.description.abstract | The 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.sponsorship | We 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.iso | eng | |
dc.publisher | Cell Press | |
dc.type.hasVersion | VoR | |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/4.0/ | * |
dc.subject | NADH-DEHYDROGENASE | |
dc.subject | BINDING PROTEINS | |
dc.subject | MESSENGER-RNAS | |
dc.subject | RIBOSOMAL-RNA | |
dc.subject | ND6 SUBUNIT | |
dc.subject | COMPLEX | |
dc.subject | TRANSLATION | |
dc.subject | DNA | |
dc.subject | POLYADENYLATION | |
dc.subject | TRANSCRIPTOME | |
dc.title | A Mitochondria-Specific Isoform of FASTK Is Present In Mitochondrial RNA Granules and Regulates Gene Expression and Function | |
dc.type | journal article | |
dc.rights.license | Attribution-NonCommercial-NoDerivatives 4.0 Internacional | * |
dc.identifier.pubmedID | 25704814 | |
dc.format.volume | 10 | |
dc.format.page | 1110-1121 | |
dc.identifier.doi | 10.1016/j.celrep.2015.01.063 | |
dc.contributor.funder | Swiss National Science Foundation | |
dc.contributor.funder | Junta de Castilla y León (España) | |
dc.contributor.funder | Roche | |
dc.description.peerreviewed | Sí | |
dc.relation.publisherversion | https://doi.org/10.1016/j.celrep.2015.01.063 | |
dc.identifier.journal | Cell Reports | |
dc.identifier.journal | Cell Rep. 2015; 10(7):1110-21 | |
dc.repisalud.orgCNIC | CNIC::Grupos de investigación::Genética Funcional del Sistema de Fosforilación Oxidativa | |
dc.repisalud.institucion | CNIC | |
dc.rights.accessRights | open access | es_ES |