Heather Durham, professor at the Montreal Neurological Institute of McGill University in Quebec, Canada, was awarded an MDA research grant totaling $355,936 over a period of three years to study the consequences of mutations in the FUS gene for muscle-controlling nerve cells called motor neurons.
Mutations in FUS are known to be a cause of amyotrophic lateral sclerosis (ALS), a disease that involves the death of motor neurons. The FUS protein binds to RNA, which cells use to bring protein-making instructions from the nucleus to other parts of the cell. FUS joins up with other proteins to do this. FUS mutations can disrupt the protein's ability to move through the cell and bind properly to other cell components to make these transport complexes.
“In neurons, the distribution of these RNA-containing complexes is particularly important for maintaining connections with other neurons, and for responding to the level of neuronal activity and stress,” Durham says. Her research will study the details of how mutations affect trafficking of FUS and its partners, including RNA, and how these abnormalities relate to or affect cellular adaptive responses. Understanding these important changes may shed light on how these and other ALS-causing gene mutations lead to the death of motor neurons.
“This is a very exciting time in ALS research,” she says. “The recent discoveries of new genes associated with ALS point us toward the commonalities and differences in the multiple disorders we call ALS. Those commonalities will not only help us to identify targets for therapy, but will establish the best preclinical models in which to evaluate their efficacy.”
Funding for this MDA grant began Feb. 1, 2013.