Wilms tumor protein

Definition:

Transcription factor that plays an important role in cellular development and cell survival. Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3'. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA.

References:

[1]. Gromoslaw A Smolen, et al. SUMO-1 modification of the Wilms' tumor suppressor WT1. Cancer Res. 2004 Nov 1;64(21):7846-51. [Content Brief]

[2]. T B Hamilton, et al. High affinity binding sites for the Wilms' tumour suppressor protein WT1. Nucleic Acids Res. 1995 Jan 25;23(2):277-84. [Content Brief]

[3]. Raphael Stoll, et al. Structure of the Wilms tumor suppressor protein zinc finger domain bound to DNA. J Mol Biol. 2007 Oct 5;372(5):1227-45. [Content Brief]

[4]. Hideharu Hashimoto, et al. Wilms tumor protein recognizes 5-carboxylcytosine within a specific DNA sequence. Genes Dev. 2014 Oct 15;28(20):2304-13. [Content Brief]

[5]. Miguel N Rivera, et al. The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activity. Proc Natl Acad Sci U S A. 2009 May 19;106(20):8338-43. [Content Brief]

[6]. Tristen C Weiss, et al. Contribution of individual amino acids to the RNA binding activity of the Wilms' tumor suppressor protein WT1. Biochemistry. 2009 Jan 13;48(1):148-55. [Content Brief]

[7]. M Andrea Markus, et al. WT1 interacts with the splicing protein RBM4 and regulates its ability to modulate alternative splicing in vivo. Exp Cell Res. 2006 Oct 15;312(17):3379-88. [Content Brief]

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