Post-transcriptional control of gene expression in human mitochondria

• Applicant: Dr. Hue-Tran Hornig-Do
• Subject Area: Cell Biology
• Term: from 2011 to 2013
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 195176819

All cells in the body require energy to function and the mitochondria form the subcompartment of all cells that is responsible for converting breakdown of food products into a usable energy form. For this reason it is critical that this organelle is healthy and can function properly. A group of diseases have been identified that arise when this organelle ceases to function correctly. These diseases can arise from a defect in a protein that is encoded on mitochondrial DNA or a protein that functions in the mitochondrion but that is encoded in the nucleus. Amongst the group of patients with mitochondrial disease, approximately one-third are affected by this specific subgroup of diseases and more commonly are children. Unfortunately we do not yet fully understand how this organelle behaves normally or which proteins are critical to maintain normal activity. By analysing cell lines derived from patients who have mitochondrial disease we can use various assays to identify which aspect of the organelle metabolism has been affected. We will also use specific techniques that should allow identification of the mutated gene, which will allow us to replace this in the cells in culture. In this way we will be able to considerably increase our understanding of both the basic mechanisms of mitochondrial gene expression in humans and also how this is affected in a large class of mitochondrial diseases.

• DFG Programme: Research Fellowships
• International Connection: United Kingdom
• Host: Dr. Zofia Chrzanowska-Lightowlers