Transcription factor hepatocyte nuclear factor 1ß controls a transcriptional network regulating cell polarity in kidney cells

Mutations or deletions in HNF1ß cause autosomal dominant tubulointerstitial kidney disease (ADTKD)-HNF1ß, a rare and heterogenous disease characterized by renal cysts and/or malformation, maturity-onset diabetes of the young, hypomagnesemia, hypokalemia and hypocalciuria. The electrolyte disturbances may develop in the distal part of the nephron, which is important for finetuning of Mg2+, Ca2+, K+ reabsorption. Moreover, this segment is a prevalent site of cyst formation in ADTKD-HNF1ß patients. Therefore, we aim to study the transcriptional network directed by HNF1ß in the distal part of the nephron. For this we combined HNF1ß ChIP-seq and mRNA expression data to identify direct targets of HNF1ß in a renal distal convoluted tubule cell line (mpkDCT). Overall design: RNA sequencing of mpkDCT cells transfected with siMock and siHNF1B, combined with ChIP-seq data from GSM2051311