Topic: untargeted metabolomics

Primary mitochondrial diseases are caused by mutations in genes that perturb mitochondrial function and frequently affect the nervous system in patients. Complex I deficiency is the most common childhood mitochondrial disease and is caused by mutations in genes encoding complex I structural or assembly components. In patients, mutations in the complex I subunit NDUFS1 typically cause severe and rapidly progressive leukoencephalopathy and death during childhood. We developed a Drosophila model of complex I deficiency using knockdown of the NDUFS1 homolog ND-75 in neurons (ND-75 KD). ND-75 KD fl