HNF-1B specifically regulates the transcription of the γa-subunit of the Na+/K+-ATPase

► Defects in HNF-1B transcription factor affect Mg2+ handling in the distal kidney. ► γa- and γb- subunits of the Na+/K+-ATPase colocalize in the distal convoluted tubule of the nephron. ► HNF-1B specifically activates γa expression. ► HNF-1B mutants have a dominant negative effect on wild type HNF-1B activity. ► Defective transcription of γa may promote renal Mg2+ wasting.