In summary, a defective engagement of mitophagy and mitochondrial biogenesis in response to ER stress triggered by the misfolded UMOD stimulates cGAS-STING signaling and innate immune response, which may eventually lead to tubular cell death and fibrosis in ADTKD-UMOD. The gene discussed is STING1; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.