Grant Details

Role of Chaperone Dysfunction in ALS
Xu, MD, PhD, Zuoshang
University of Massachusetts, Worcester, MA

Project Summary
Molecular chaperones are proteins that help to protect the structure and function of other cellular proteins. They help cells to survive and function despite such stresses as heat or chemical shocks. Motor neurons naturally express relatively low levels of the chaperones. This makes them particularly susceptible to stress. Xu and colleagues will see if the scarcity of chaperones in motor neurons is what makes these cells so susceptible to damage in ALS. The reduction in chaperones can be detected several months before the clinical disease begins. The scientists seek the exact chaperone that interacts with mutant SOD1, so that future experiments can specifically manipulate it to alter the disease (see also SOD1 and ALS).