PAX8 and autosomal dominant polycystic kidney disease: While tissue-specific deletion is a genetically accurate tool (KSP and Pax8 drivers), ADPKD in humans is a disease that affects multiple organs, not only the kidneys, and therefore analysis of results from kidney-specific models should be performed with caution and in combination with hypomorphic mutants such as the Pkd1nl/nl used in this study.