Cardiac mitochondrial function depends on BUD23 mediated ribosome programming

• DOI: 10.7554/elife.50705
• Publication Date: 2020-01-15T13:01:07Z
• Authors: Matthew Baxter; Maria Voronkov; Toryn Poolman; Gina Galli; Christian Pinali; Laurence Goosey; Abigail Knight; Karolina Krakowiak; Robert Maidstone; Mudassar Iqbal; Min Zi; Sukhpal Prehar; Elizabeth J Cartw...; Julie Gibbs; Laura C Matthews; Antony D Adamson; Neil E Humphreys; Pedro Rebelo-Guiomar; Michal Minczuk; David A Bechtold; Andrew Loudon; David Ray
• Keywords: Male; 0301 basic medicine; Cell biology; Mouse; QH301-705.5; cardiac; Science; Mitochondrial Proteins; Mice; 03 medical and health sciences; cell biology; Animals; Humans; Myocytes, Cardiac; human; Protein Interaction Maps; Biology (General); mouse; protein translation; 0303 health sciences; Base Composition; Protein translation; Q; R; Cell Biology; Methyltransferases; Embryo, Mammalian; Ribosome; Mitochondria; 3. Good health; mitochondria; ribosome; A549 Cells; Medicine; Female; 5' Untranslated Regions; Cardiomyopathies; Cardiac; Ribosomes; Research Article; Human

Efficient mitochondrial function is required in tissues with high energy demand such as the heart, and dysfunction associated cardiovascular disease. Expression of proteins tightly regulated response to internal external stimuli. Here we identify a novel mechanism regulating content function, through BUD23-dependent ribosome generation. BUD23 was for maturation, normal 18S/28S stoichiometry modulated translation transcripts human A549 cells. Deletion Bud23 murine cardiomyocytes reduced leading severe cardiomyopathy death. We discovered that selectively promotes ribosomal interaction low GC-content 5'UTRs. Taken together critical role bioenergetics gene expression, by promoting efficient mRNA 5'UTR GC content. emerges essential mouse development, postnatal cardiac function.