How nerve cells are damaged by accumulation of abnormal proteins
The findings, which appear in the journal eLife , may one day lead to better therapies and treatments for these diseases. The prion protein plays a crucial role in fatal neurodegenerative disorders like Creutzfeldt-Jakob disease in humans and "mad cow disease" in cattle. Prion diseases are part of larger group of human neurodegenerative disorders, including Alzheimer's, Parkinson's and Huntington's diseases, which are all due to the abnormal accumulation of protein aggregates in the brain. According to the researchers, how nerve cells are damaged in these diseases has remained a mystery until now. "Our work shows that the prion protein acts like a molecular on-off switch. In the "on" positon, one end of the protein delivers a toxic signal to nerve cells, while in the "off" position, the other end of the protein serves as a brake to reduce the toxic signal. Moreover, copper, a metal that is a normal component of brain biochemistry, ...