WT1 and Wilms’ Tumor Sarah DeHart. DNA-binding transcription factor Protein carries 4 C-terminal zinc fingers 36 different isoforms based on splicing.

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Presentation transcript:

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 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 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 8c19-11e2-af4f-00000aab0f26&acdnat= _bea33c28750fe3929effc614e36c2d31 Gessler, M. (1991). WT1 (Wilms’ tumor suppressor gene). Retrieved from Hohenstein, P., & Hastie, N. D. (2006). The many facets of the Wilms’ tumour gene, WT1. Human Molecular Genetics,15(2), R196–R201. doi: /hmg/ddl196 Miller-Hodges, E., & Hohenstein, P. (2011). WT1 in disease: shifting the epithelial–mesenchymal balance. Journal of Pathology, 226, 229–240. doi: /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 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, Retrieved from Tiller, G. E. (2011). Wilms Tumor 1; WT1. Retrieved from WT1. (2009). Retrieved from Pictures – – – –