Research Summary

Dr. Todi's research focuses on two areas. One is the understanding of mechanisms that lead to degeneration in the most common, dominantly inherited ataxia in the world, Spinocerebellar Ataxia Type 3 (SCA3). By using a combination of Drosophila melanogaster genetics, in vitro biochemistry and mammalian cell biology, the Todi laboratory has found new clues into the life cycle of the causative protein in SCA3, ataxin-3: how it acts as an enzyme, its functions, the manner in which it is disposed off in the cell, and what regulates its toxicity in SCA3. Current studies are aimed at understanding how partners of ataxin-3 control its toxicity and how these interactions can be prevented for the purposes of therapy. The other of research in the Todi lab area focuses on general mechanisms of ubiquitin biology. Ubiquitin (Ub) is a small protein whose chemical conjugation to other proteins regulates their function and fate. The Todi lab has made use of fruit fly genetics and in vitro biochemistry to understand how this vital protein is regulated and recycled in the cell.