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

Mitochondrial DNA (mtDNA) replication and transcription are essential for 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 requirement of PPR proteins in RNA metabolism has not been fully examined. In this paper, we focus on the homolog of ( ), encoded by the gene. A loss-of-function mutant of is lethal during the second instar. In addition, mutants exhibit reduced expression of a group of genes related to mitochondrial function and ribosome biogenesis, and conversely, they show up-regulated expression of neuronal development-related genes. These results suggest that has important functions in relation to mtDNA and is essential for development.