This research focuses on primary Distal Renal Tubular Acidosis (dRTA), a rare childhood-onset kidney disorder, causing metabolic acidosis and renal damage. About 60% of cases are linked to a single gene disorder, leaving potential new disease genes to be discovered. In genetically unsolved dRTA patients, the scientists identified variants in WDR72 as a potential novel dRTA cause. Here, they aim to define a role for WDR72 in normal renal function but also in dRTA pathogenesis. Therefore, Wdr72 deficient mice will be analyzed for a dRTA condition and the role of WDR72 in cell culture and organoids by analyzing acidification and protein interactions will be explored. Overall, this project aims to uncover molecular mechanisms of WDR72 in dRTA using mouse, cell and organoid models.