Franco Pradelli

      Ritratto di Franco Pradelli

Neuroscience, Technology and Society, XXXVII series 

Grant sponsor
Fondazione AIRC

Silvio Tosatto



Project description

The von Hippel-Lindau (VHL) syndrome is a hereditary disease associated with highly vascularized tumors and cysts arising in multiple organs, from the eye to the kidney. We know that the root cause of the disease is the genetic mutation of a single protein, called von Hippel-Lindau protein (pVHL), which plays a pivotal role in cellular oxygen sensing. However, the high variability observed in the disease progress in different patients suggests that there is more to discover on the function of this protein, especially considering its role in cancer. While experimental and computational studies are providing further information on the disease at the molecular level, not many tools have been able to couple these data with the actual effect of the disease on the patients’ organs. Mathematical models can fill this gap, providing a way to predict VHL-cancer progression based on the putative effect of the pVHL mutations in a tissue-specific manner. Moreover, mathematical modeling can handle many different aspects of tumor development, like the interaction of the tumor with the host tissue, with the extracellular matrix (ECM), and with the blood vessels, providing a more complete description of the disease. Thus, my research will focus on the use of these models to describe and predict VHL-tumor progression, potentially shedding new light on the role of pVHL in cancer and developing a tool to predict the disease progression.