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oai:repisalud.isciii.es:20.500.12105/55132024-10-31T11:55:21Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605

00925njm 22002777a 4500 dc Jourdain, Alexis A. author Koppen, Mirko author Rodley, Christopher D. author Maundrell, Kinsey author Gueguen, Naig author Reynier, Pascal author Guaras, Adela author Enriquez, Jose Antonio author Anderson, Paul author Simarro, Maria author Martinou, Jean-Claude author 2015 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. 2211-1247 http://hdl.handle.net/20.500.12105/5513 25704814 10.1016/j.celrep.2015.01.063 Cell Reports Cell Rep. 2015; 10(7):1110-21 NADH-DEHYDROGENASE BINDING PROTEINS MESSENGER-RNAS RIBOSOMAL-RNA ND6 SUBUNIT COMPLEX TRANSLATION DNA POLYADENYLATION TRANSCRIPTOME A Mitochondria-Specific Isoform of FASTK Is Present In Mitochondrial RNA Granules and Regulates Gene Expression and Function