Regulation of the translation and turnover of mRNAs is central to the control of gene expression. Eukaryotes have evolved mechanisms to sequester mRNAs and their associated RNA-binding proteins into non-membrane delimited bodies called RNA granules as a mechanism to control these processes and to respond to changing cellular demands and physiological stresses. We have identified mitochondrial RNA granules and find that they contain a large toolbox of proteins dedicated to RNA metabolism including proteins involved in transcript processing, rRNA and tRNA modification, mRNA turnover, and ribosome biogenesis. I will discuss recent developments and the relevance to mechanisms of mitochondrial disease.
Dr. Eric Shoubridge is James McGill Professor and Chair of the Department of Human Genetics at McGill University. He obtained his B.Sc. and his M.Sc. from McGill University, and completed his Ph.D. at the University of British Columbia in 1981. He conducted his post-doctoral training in the Biochemistry Department of Oxford University and joined the faculty at the MNI in 1985. Research in his laboratory focuses on the molecular genetics of mitochondrial disease.