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

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

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MRC Institute of Genetics and Molecular Medicine, University of Edinburgh, Western General Hospital, Crewe Road (S), Edinburgh, EH4 2XU, UK


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.


Developmental disorders; Homeostasis; Molecular mechanisms of disease; WT1

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