To directly demonstrate that STING pathway is activated by cytosolic leakage of mtDNA and recapitulate the in vivo findings in our mouse model, we generated a cellular model of ADTKD-UMOD in HEK 293 cells by transducing the cells with lentivirus expressing WT or mutant UMOD (H177-R185 del) fused to N-terminal GFP. This evidence concerns the gene STING1 and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.