The abundance of LONP1, a mitochondrial protease involved in degradation of damaged proteins and in mitochondrial quality control under stress conditions24, 25, was increased in ADTKD-UMOD suggesting the occurrence of stressed mitochondria, which might be more susceptible to enter the mitophagy process. The gene discussed is LONP1; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.