In the current study, we also show that changes in glutamine, glutamate, aspartate, and asparagine can be observed in the cystic PKD kidneys, and corrected upon silencing of ASNS in vivo, demonstrating that the changes in levels of circulating metabolites in the ADPKD population are likely the result of their change in the kidneys, possibly also in patients. Here, ASNS is linked to autosomal dominant polycystic kidney disease.