We are investigating the clinical features and molecular mechanisms leading to neurological failure with COX deficiency in a mouse knockout model of Apopt1/Coa8 deficiency. We have established a collaboration with the MRC-Mitochondrial Biology Unit (MBU) mass spectrometry core facility and with the lab of Prof. Eric Shoubrdge (McGill University, Montreal, CA) to identify possible partners of Apopt1 by using SILAC and BioID technologies. We are also studying COA8 regulation via redox signalling in collaboration with Prof. Michael P. Murphy (MBU).