Wilms' tumour 1 (WT1) in development, homeostasis and disease

Development. 2017 Aug 15;144(16):2862-2872. doi: 10.1242/dev.153163.

Abstract

The study of genes mutated in human disease often leads to new insights into biology as well as disease mechanisms. One such gene is Wilms' tumour 1 (WT1), which plays multiple roles in development, tissue homeostasis and disease. In this Primer, I summarise how this multifaceted gene functions in various mammalian tissues and organs, including the kidney, gonads, heart and nervous system. This is followed by a discussion of our current understanding of the molecular mechanisms by which WT1 and its two major isoforms regulate these processes at the transcriptional and post-transcriptional levels.

Keywords: Developmental disorders; Homeostasis; Molecular mechanisms of disease; WT1.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Evolution, Molecular
  • Homeostasis
  • Humans
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / metabolism*
  • Protein Isoforms / genetics
  • Protein Isoforms / metabolism
  • WT1 Proteins / genetics
  • WT1 Proteins / metabolism
  • Wilms Tumor / genetics
  • Wilms Tumor / metabolism*

Substances

  • Protein Isoforms
  • WT1 Proteins