Role of redox signalling on mtDNA expression and regulation
The project aims at defining the impact of redox signalling in mitochondrial patient cell lines on the regulation of mtDNA expression and regulation. The project will be based on the use of high-throughput and high content approaches in cultured cells. The focus will be on redox modifications of mitochondrial proteins in disease vs healthy states and on the impact of Khondrion compound library of antioxidants/redox modulators on their functionality.
Iannetti E, Smeitink J, Beyrath J, Willems P, Koopman W. Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy. Nat Protoc. 2016 Sep;11(9):1693-710
Koopman W, Beyrath J, Fung C-W, Koene S, Rodenburg R, Willems P, Smeitink J. Mitochondrial disorders in children: toward development of small-molecule treatment strategies. EMBO Mol Med. 2016 Mar 31;8(4):311-27. doi: 10.15252/emmm.201506131