The data now reported confirm that also the RNF213 protein abundance is upregulated > 4-fold in ClpP−/− brain, while Lonp1 as the other AAA+ ATPase/peptidase in the mitochondrial matrix and Tfam as the main mitochondrial transcription factor show unchanged levels (Fig. 1), indicating a quite selective effect of ClpP for Rnf213. This ClpP-genotype-dependent impact on Rnf213 now was documented also for immune cells, namely BMDM upon challenge with LPS rather than Pam3CSK4, both of them being simulators of bacterial infection (Fig. S1). This evidence concerns the gene LONP1 and bacterial infectious disease.