To gather a comprehensive picture of the metabolic derangements observed in ADPKD mouse models, we applied non-targeted global metabolomics25 to an orthologous mouse model of ADPKD carrying inactivation of the Pkd1 gene exclusively in the kidney as to avoid confounding effects derived from extra-renal inactivation. This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.