Research Grant Award

Cláudio M. Gomes, PhD

Instituto Tecnologia Química e Biológica., Universidade Nova de Lisboa, Portugal
Frataxin folding, chaperone role and interactions with iron-sulfur biosynthesis proteins: contributions to understand Friedreich's ataxia

The neurodegenerative disease Friedreich ataxia (FRDA) is the most common of hereditary ataxias with an estimated prevalence of 1 in 50000 individuals. This pathology results from a reduction in frataxin levels, a small protein localized in the on a cellular organelle (mitochondrion). Frataxin function is not yet fully understood but previous data shows a role in the formation of iron-sulfur clusters, the inorganic cofactors that will be incorporated into many different proteins and are essential for life. A more precise understanding of the biological function of frataxin is necessary in order to better understand how FRDA is developed. The present project focuses on the study of the interaction between frataxin and other proteins involved in the biosynthesis of iron-sulfur clusters, using genetic, biochemical and biophysical methodologies.