Protein Misfolding and Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
Dr. Bosque is investigating the role of misfolded and aggregated proteins in amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease. ALS is a fatal disease that causes progressive weakness by killing off motor neurons. The root cause of ALS is unknown. Microscopic examination of the spinal cord and brain of people who die of ALS shows signs of some protein building up in misfolded and abnormally aggregated form. This misfolded protein may be the cause of ALS and the goal of Dr. Bosque's investigations is to identify the protein and why it misfolds. Current investigations are pursuing the possibility that this protein may misfold in a manner analogous that of the prion protein, which causes Creutzfeldt-Jakob disease, mad cow disease and other conditions. The peculiar property of the prion protein is that it can be triggered to misfold by the presence of already misfolded prion protein, so that misfolding can propagate almost like an infection. A goal is to exploit this hypothetical property of the protein causing ALS to identify it and its mechanism of misfolding.