Our crucial novel insight of ClpP-dependent GFM1 and GRSF1 abundance therefore suggests a common pathway for Perrault syndrome pathogenesis, since the genetic causes of autosomal recessive Perrault syndrome include variants in the mitochondrial tRNA amino acid synthetases HARS2 and LARS2, the mitochondrial tRNA processing enzyme PRORP, the mitochondrial DNA/RNA helicase/primase TWNK, and the peptidase ClpP. Here, TWNK is linked to Perrault syndrome.