LRPPRC and Leigh syndrome: While defects in some Leigh syndrome-associated genes do indeed show increased ROS, including sicily, disruption of the fly ortholog of LRPPRC [Leucine-rich PPR motif-containing protein, mitochondrial, Lrpprc2 (also referred to as ppr)] did not result in a significant increase in ROS levels despite mutant photoreceptors undergoing neurodegeneration (Jaiswal et al., 2015).