Parathyroid Hormone Receptor 1 Facilitates Cyst Growth in Genetic Models of Autosomal Dominant Polycystic Kidney Disease

The authors investigate the role of parathyroid hormone receptor 1 (Pth1r) in promoting cyst growth in autosomal dominant polycystic kidney disease (ADPKD) models, aiming to identify specific ciliary receptors that mediate extracellular signals contributing to cyst progression. Their findings reveal that Pth1r is upregulated in early cystic kidneys, localizes to primary cilia, and its genetic inactivation significantly reduces cyst growth, suggesting it acts as a ciliary GPCR that links parathyroid hormone signaling to cyst-promoting pathways in ADPKD. Additionally, treatment with the calcimimetic cinacalcet was shown to alleviate cystic burden and normalize PTH levels, highlighting its potential therapeutic relevance.