Role of transcription factor hepatocyte nuclear factor-1β in polycystic kidney disease

Research output: Contribution to journalArticlepeer-review

Abstract

Hepatocyte nuclear factor-1β (HNF-1β) is a DNA-binding transcription factor that is essential for normal kidney development. Mutations of HNF1B in humans produce cystic kidney diseases, including renal cysts and diabetes, multicystic dysplastic kidneys, glomerulocystic kidney disease, and autosomal dominant tubulointerstitial kidney disease. Expression of HNF1B is reduced in cystic kidneys from humans with ADPKD, and HNF1B has been identified as a modifier gene in PKD. Genome-wide analysis of chromatin binding has revealed that HNF-1β directly regulates the expression of known PKD genes, such as PKHD1 and PKD2, as well as genes involved in PKD pathogenesis, including cAMP-dependent signaling, renal fibrosis, and Wnt signaling. In addition, a role of HNF-1β in regulating the expression of noncoding RNAs (microRNAs and long noncoding RNAs) has been identified. These findings indicate that HNF-1β regulates a transcriptional and post-transcriptional network that plays a central role in renal cystogenesis.

Original languageEnglish (US)
Article number109568
JournalCellular Signalling
Volume71
DOIs
StatePublished - Jul 2020

Bibliographical note

Funding Information:
We thank Karam Aboudehen for critically reviewing the manuscript. We apologize to colleagues whose work we were unable to cite because of space constraints. Research from the authors' laboratory was supported by the National Institute of Diabetes, Digestive and Kidney Diseases of the National Institutes of Health under award number R37DK042921.

Funding Information:
We thank Karam Aboudehen for critically reviewing the manuscript. We apologize to colleagues whose work we were unable to cite because of space constraints. Research from the authors' laboratory was supported by the National Institute of Diabetes , Digestive and Kidney Diseases of the National Institutes of Health under award number R37DK042921 .

Publisher Copyright:
© 2020 Elsevier Inc.

Keywords

  • Chromatin immunoprecipitation
  • Cyclic AMP
  • Gene transcription
  • Kidney cysts
  • Kidney development
  • Kidney fibrosis
  • Noncoding RNA
  • Wnt signaling

PubMed: MeSH publication types

  • Journal Article
  • Research Support, N.I.H., Extramural

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