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Commentary

Exploring prion protein biology in flies: Genetics and beyond

Diego E. Rincon-Limas, Sergio Casas-Tinto and Pedro Fernandez-Funez

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The fruit fly Drosophila melanogaster has been a favored tool for genetic studies for over 100 years and has become an excellent model system to study development, signal transduction, cell biology, immunity, and behavior. The relevance of Drosophila to humans is perhaps best illustrated by the fact that more than 75% of the genes identified in human diseases have counterparts in Drosophila. During the last decade, many fly models of neurodegenerative disorders have contributed to the identification of novel pathways mediating pathogenesis. However, the development of prion disease models in flies has been remarkably challenging. We recently reported a Drosophila model of sporadic prion pathology that shares relevant features with the typical disease in mammals. This new model provides the basis to explore relevant aspects of the biology of the prion protein, such as uncovering the genetic mechanisms regulating prion protein misfolding and prion-induced neurodegeneration, in a dynamic, genetically tractable in vivo system.

Authors

Diego E. Rincon-Limas Corresponding author: derincon@utmb.edu

University of Texas Medical Branch

Sergio Casas-Tinto

CNIO

Pedro Fernandez-Funez Corresponding author: pedro.fernandez@neurology.ufl.edu

University of Florida

This is an open-access article

 Download PDF

If the document does not open, please right-click on the link (control-click on a Macintosh) and select the option to save the file to disk.