It was interesting to note that a proteomics approach comparing human ARPKD kidneys and control kidneys identified upregulation of many proteins localizing to mitochondria (57) and that an increase of the oxygen consumption rate was noted in HEK293 cells with truncating PKHD1 variants whereas a reduction of mitochondrial mass has been reported in cyst-lining cells from a murine Pkd1 model. This evidence concerns the gene PKD1 and autosomal recessive polycystic kidney disease.