Glerum, D. Moira - University of Waterloo

个人简介

The research in the Glerum lab is geared to understanding the molecular bases for inherited diseases that affect the function of mitochondria, our cellular ‘power plants’. Through the use of the yeast model system, our studies have identified previously unknown proteins required to generate functional mitochondria and allowed us to improve our understanding of the roles of these proteins in human disease. Physiology, Cell and Developmental Biology Molecular Genetics Microbiology American Society for Microbiology American Society for Biochemistry and Molecular Biology Canadian Society of Molecular BioSciences 1990 PhD Biochemistry, University of Toronto 1984 BSc Biochemistry, University of Toronto

研究领域

Mitochondria are essential energy generators in almost all eukaryotic cell and defects in mitochondrial structure and function have been identified in a wide variety of human diseases. In fact, defects in mitochondrial function may be the most common underlying cause of neurodegenerative disease! Mitochondria are comprised of proteins encoded in two different genomes - the nuclear (nDNA) and the mitochondrial (mtDNA). Our lab is studying the contributions of both of these genomes to neurodegenerative diseases and cancer. The nuclear genome encodes most of the proteins required for mitochondrial formation. One of the key enzymes found in mitochondria is cytochrome oxidase (COX), which consists of 13 subunits - 3 encoded in mtDNA and 10 encoded in the nucleus. In addition, there are some two dozen proteins involved in ensuring that COX is correctly assembled, all of which are also encoded in the nDNA. The COX assembly pathway is most often defective in human COX deficiencies, which are the most common of the mitochondrial respiratory chain disorders. These diseases usually present early in life and are almost always fatal. The COX assembly pathway is still only partially understood and we are using yeast as a model system for delineating this process. Our studies are furthering our understanding of how mutations in COX assembly genes result in fatal neurological disease. Microfluidic chip, or lab-on-a-chip, technologies have the potential to revolutionize both health care delivery and biomedical research. Given the ever-increasing list of disorders with a documented mitochondrial dysfunction, technologies that would enable us to investigate mitochondrial function at the level of a single cell would further our understanding of the contributions of this organelle to disease pathologies. In collaboration with the Backhouse lab in Electrical and Computer Engineering, we are therefore also developing microfluidic chip-based assays for use in studying mitochondrial disease.

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Hall, G.H., Glerum, D.M., Backhouse, C.J. (2016) A light emitting diode, photodiode-based detection system for DNA analysis with microchip electrophoresis. Electrophoresis 37, 406-413. Hall, G.H., Sloan, D., Ma, T., Couse, M.H., Martel, S., Elliott, D.G., Glerum, D.M., Backhouse, C.J. (2014) An optical relay approach to very low cost hybrid polymer-CMOS electrophoresis instrumentation. J. Chrom. A 1349, 122-128. Veniamin, S., Sawatzky, L.G., Banting, G., Glerum, D.M. (2011) Characterization of the peroxide sensitivity of COX deficient yeast strains reveals unexpected relationships between COX assembly proteins. Free Radic. Biol. Med. 51, 1589-1600.