2026-04-29T04:52:19Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/55132024-10-31T11:55:21Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605
Repisalud
author
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
funder
Swiss National Science Foundation
funder
Junta de Castilla y León (España)
funder
Roche
2017-12-01T07:37:25Z
2017-12-01T07:37:25Z
2015
2211-1247
http://hdl.handle.net/20.500.12105/5513
25704814
10.1016/j.celrep.2015.01.063
Cell ReportsCell Rep. 2015; 10(7):1110-21
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.
eng
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
journal article
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AMitochondria-SpecificIsoformOfFASTK_2015
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AMitochondria-SpecificIsoformOfFASTK_2015_Table
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AMitochondria-SpecificIsoformOfFASTK_2015_Supp1.txt
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AMitochondria-SpecificIsoformOfFASTK_2015_Supp1.txt
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AMitochondria-SpecificIsoformOfFASTK_2015.txt
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AMitochondria-SpecificIsoformOfFASTK_2015_Table.txt