Prion-like Activity in Neurodegenerative Disorders

Could misfolded human proteins with prion-like activity contribute to the progression of certain chronic diseases such as Alzheimer’s, Parkinsons, and Huntington’s disease? A common feature of all three of these diseases is the presence of abnormal accumulations of certain misfolded proteins in or around nerve cells in the brain. Eventually these protein accumulations become so extensive that they choke off nerve cell function.

No one is saying that these diseases are infectious, like mad cow disease. But according to the latest thinking, once an endogenous protein "goes rogue" and misfolds, it might then cause nearby normal proteins to misfold as well. Once the process starts it could become self-propagating, from one region of the brain to the next.