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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, ...

New 'GPS' neuron discovered

Every day billions of people across the planet successfully navigate their environments, for example when they go to work or head home. Such journeys generally happen with little conscious effort and rest on the brain's ability to use overall knowledge of an environment to make estimates of where it finds itself. The ability to make fine grained assessments of location is seated in the hippocampus, a seahorse-shaped structure located in the temporal lobe. Research shows that the precise mechanism for navigation includes hippocampal place cells, which increase or decrease in electrical activity depending on one's location. However, when making their daily commute, people don't need very detailed representations of which houses they pass in which order. Instead, they can make due with more course information. Left at the museum and somewhere down the road right again at the supermarket, called topographical orientation. Building on current research, the researchers invest...