An international team of researchers has identified the misfolded protein that causes both amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, and a less well-known condition, frontotemporal lobar degeneration, according to Virginia Lee, Ph.D., of the University of Pennsylvania.
It is TAR DNA-binding protein 43 (TDP-43), which has several functions and is found in the nucleus of many cell types, Dr. Lee and colleagues reported in the Oct. 6 issue of Science.
Misfolded proteins are a common motif in neurodegenerative diseases. They are tagged for recycling by the protein ubiquitin, but instead of being broken down they are dumped in the neurons. Yet the specific ubiquitinated protein involved in ALS and frontotemporal lobar degeneration had not been identified previously, Dr. Lee said. The finding "resolves a long-standing enigma," she and colleagues noted.
Awareness in Presumed Vegetative State?
