The Yeast Connection to Friedreich Ataxia

There have been both genetic and biochemical advances in the understanding of inherited neurodegenerative diseases, such as Huntington disease (HD) and Friedreich ataxia (FA). In FA, these advances have come from the following two approaches: the mapping of the disease gene in humans, and work with a distantly related model organism, Saccharomyces cerevisiae. These two approaches converged, with the study of the human gene leading to the yeast homologue and the study of the yeast mutant phenotypes leading to the human homologue and its associated disease.