Hepatocyte nuclear factor 1 homeobox B (HNF1β) is an essential transcription factor for the development and functioning of the kidney. Mutations in HNF1β cause autosomal dominant tubulointerstitial kidney disease. The transient receptor potential melastatin type 6 (TRPM6) epithelial Mg(2+) channels participate in transcellular Mg(2+) transport in the kidney and intestine. Previous reports suggested a hormonal control of TRPM6 activity. Fine-tuning of renal calcium (Ca2+) reabsorption takes place in the late distal convoluted and connecting tubules. Congenital anomalies of the kidney and urinary tract (CAKUT) constitute one of the most common developmental diseases in humans; however, the cause for most patients remains unknown. Efforts to understand the molecular mechanisms underlying CAKUT are ongoing. The study of these mechanisms will provide valuable insights into the development and functioning of the kidney.