WT1 and Wilms’ Tumor
Sarah DeHart
DNA-binding transcription factor
Protein carries 4 C-terminal zinc fingers
36 different isoforms based on splicing
Specific isoforms can have different functions
WT1 Expression in Development
WT1 Pathway
WT1 Role in EMT/MET
WT1 inheritance patters
What’s wrong here?
References
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Chau, Y.-Y., & Hastie, N. D. (2012). The role of Wt1 in regulating mesenchyme in cancer, development, and tissue
homeostasis. Trends in Genetics, 28(10), 515–524. Retrieved from
http://www.sciencedirect.com/science/article/pii/S0168952512000686
Haber, D. A., Buckler, A. J., Glaser, T., Call, K. M., Pelletier, J., Sohn, R. L., Douglass, E. C., et al. (1990). An Internal
Deletion within an 11p13 Zinc Finger Gene Contributes to the Development of Wilms’ Tumor. Cell, 61, 1257–1269.
Retrieved from http://ac.els-cdn.com/009286749090690G/1-s2.0-009286749090690G-main.pdf?_tid=305254228c19-11e2-af4f-00000aab0f26&acdnat=1363205238_bea33c28750fe3929effc614e36c2d31
Gessler, M. (1991). WT1 (Wilms’ tumor suppressor gene). Retrieved from
http://atlasgeneticsoncology.org/Genes/WT1ID78.html
Hohenstein, P., & Hastie, N. D. (2006). The many facets of the Wilms’ tumour gene, WT1. Human Molecular
Genetics,15(2), R196–R201. doi:10.1093/hmg/ddl196
Miller-Hodges, E., & Hohenstein, P. (2011). WT1 in disease: shifting the epithelial–mesenchymal balance. Journal
of Pathology, 226, 229–240. doi:10.1002/path.2977
Pritchard-Jones, K., Flemming, S., Hastie, N., Bickmore, W., Porteous, D., Godsen, C., Bard, J., et al. (1990). The
candidate Wilms’ tumour gene involved in genitourinary development. Nature, 346, 194–197. Retrieved from
http://www.nature.com.libproxy.lib.unc.edu/nature/journal/v346/n6280/pdf/346194a0.pdf
Stoll, R., Lee, B. M., Debler, E. W., Laity, J. H., Wilson, I. A., Dyson, H. J., & Wright, P. E. (2007). Structure of the
wilms tumor suppressor zinc finger domain bound to DNA. Journal of Molecular Biology, 372, 1227. Retrieved
from http://www.ncbi.nlm.nih.gov/Structure/mmdb/mmdbsrv.cgi?uid=62809
Tiller, G. E. (2011). Wilms Tumor 1; WT1. Retrieved from http://omim.org/entry/194070
WT1. (2009). Retrieved from http://ghr.nlm.nih.gov/gene/WT1
Pictures
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http://www.projekt-koni.de/77.html?&L=1
http://radiology.rsna.org/content/early/2012/11/20/radiol.12120670/F1.expansion.html
http://practicalimaging.blogspot.com/2012/07/new-case-of-week.html
http://www.sciencedirect.com/science/article/pii/S0031395505705307