ESN stimulation grant for Jessica Nouws

Nouws , Jessica

Jessica Nouws, a PhD student in the group of Leo Nijtmans, has received a stimulation grant from the Dutch hereditary metabolic diseases association (ESN) to spend on a work visit abroad. This grant enables Jessica to investigate a regulatory step in mitochondrial complex I assembly. She will perform this research in the laboratory of Robert Lightowlers in Newcastle.

One in every 5000 children/newborns are affected with hereditary mitochondrial disorders caused by a defect in the respiratory chain. These children are generally diagnosed with severe brain, heart and or muscle problems in the first two years of their life. These features often evolve progressively and are fatal since no therapy or cure is available. One fourth of these children suffers from complex I deficiency: the entry point and biggest complex of the respiratory chain does not function optimally. The components of this complex, 45 subunits, are encoded by mitochondrial and nuclear DNA. The assembly of this large protein complex is evolutionary conserved: the different functional modules are pre-assembled before they are combined, together with some additional subunits to form fully functional complex I. This process is aided by assembly factors or chaperones, of which a dozen were indentified recently. Nevertheless not much is known about the assembly of this huge and essential complex. Leo Nijtmans' group showed in 2007 that the assembly starts by constructing the hydrogenase module. This module is entirely composed of nuclear encoded subunits, to continue the assembly process the incorporation of a mitochondrial encoded subunit is required. Which makes us wonder: "how does the cell coordinate the combination of mitochondrial and nuclear encoded subunits". This is the question Jessica will address during her work visit to Newcastle.

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