WDR72 (WD repeat domain 72) is a multifunctional protein with critical roles in both enamel development and renal acid-base homeostasis. Primarily, WDR72 plays a major role in tooth enamel formation, specifically during the maturation phase of amelogenesis 1. The protein localizes to the Golgi apparatus via a C-terminal CAAX motif and regulates endocytosis of vesicular transport and microtubule assembly necessary for membrane mobilization 23. This trafficking function is essential for clearance of enamel proteins and localization of the calcium transporter SLC24A4 to ameloblast cell membranes. WDR72 mutations cause amelogenesis imperfecta, characterized by brown-discolored, rough enamel with early loss after eruption 3. Beyond dental development, WDR72 is also involved in renal acid-base regulation; inherited forms of distal renal tubular acidosis (dRTA) are caused by WDR72 variants 45. Patients with WDR72-associated dRTA present with enamel hypoplasia, nephrolithiasis, nephrocalcinosis, and renal cysts 6, reflecting WDR72's expression in multiple organ systems including kidney, brain, liver, and heart 2. Clinically, recognizing WDR72-related defects is crucial for appropriate management of both dental and renal complications.