Clotilde Lagier-Tourenne, a postdoctoral fellow at the University of California, San Diego, in La Jolla, was awarded an MDA development grant totaling $180,000 over a period of three years to study the roles of two proteins, TDP43 and FUS, in ALS (amyotrophic lateral sclerosis, or Lou Gehrig's disease).

ALS-causing mutations in the genes for two RNA binding proteins, TDP43 and FUS, appear to cause disruption in the processing of RNA (the chemical step that directs protein synthesis).

In a research mouse model, Lagier-Tourenne and colleagues have identified RNAs that TDP43 normally binds to, as well as abnormalities in the processing of RNA that occur when TDP43 is depleted.

Similarly, in their new work, the researchers plan to determine the role of FUS in the regulation of RNA in the mouse central nervous system.

The team's proposed set of studies is expected to identify a set of alterations to normal RNA processing that will define a TDP43- and FUS-dependent ALS disease signature.

"I am really grateful for the support from MDA," Lagier-Tourenne said. "It represents strong encouragement at this stage of my career and will allow me to extend my research on the role of RNA processing in neurodegeneration."

Funding for this MDA grant began August 1, 2011.