A Drosophila melanogaster ortholog of pentatricopeptide repeat domain 3 (PTCD3) gene is essential for development

Mitochondrial DNA (mtDNA) replication and transcription are critical to cellular energy metabolism. It has been suggested that pentatricopeptide repeat (PPR) proteins regulate various aspects of mitochondrial RNA metabolism, including transcription, processing, maturation and stability, and protein synthesis. However, an in vivo requirement of PPR proteins in RNA metabolism has not been fully examined. In this paper, we focus on the Drosophila melanogaster homolog of PPR domain 3 (PTCD3), encoded by CG4679 gene. A loss of function mutant of PTCD3 is lethal at the second-instar larval stage and exhibits an altered expression of a group of genes related to mitochondrial function and neuronal development. These results suggest PTCD3 has important roles in mtDNA and is essential for development.