WASHINGTON (Dispatches) -- Case Western Reserve University researchers studying prions -- misfolded proteins that cause lethal incurable diseases -- have identified the surface features of human prions responsible for their replication in the brain.
Human prions can bind to neighboring normal proteins in the brain, and cause microscopic holes. In essence, they turn brains into sponge-like structures and lead to dementia and death. These discoveries led to the ongoing scientific debate on whether prion-like mechanisms may be involved in the origin and spread of other neurodegenerative disorders in humans.
“Human prion diseases are conceivably the most heterogenous neurodegenerative disorders, and a growing body of research indicates that they are caused by distinct strains of human prions,” Jiri Safar, professor of pathology, neurology and neurosciences at the Case Western Reserve School of Medicine said. “However, the structural studies of human prions have lagged behind the recent progress in rodent laboratory prions, in part because of their complex molecular characteristics and prohibitive biosafety requirements necessary for investigating disease which is invariably fatal and has no treatment.”