- A Nobel Prize–winning discovery found that mad cow and related infectious diseases occur when aberrant proteins—prions—wreak havoc by causing normal versions of those proteins to become malformed.
- Prionlike disease processes also appear to be at work in major neurodegenerative disorders, including Alzheimer's, Parkinson's and Lou Gehrig's, although they are not transmitted from person to person.
- How proteins contort into a form that causes others to undergo a similar transformation may lead to new approaches to preventing and treating some of the world's leading neurological illnesses.
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Under a microscope, a pathologist searching through the damaged nerve cells in a brain tissue sample from a patient who has died of Alzheimer's disease can make out strange clumps of material. They consist of proteins that clearly do not belong there. Where did they come from, and why are there so many of them? And most important, what do they have to do with this devastating and incurable disorder? The search for answers has turned up a startling discovery: the clumped proteins in Alzheimer's and other major neurodegenerative diseases behave very much like prions, the toxic proteins that destroy the brain in mad cow disease.
Prions are misshapen yet durable versions of proteins normally present in nerve cells that cause like proteins to misfold and clump together, starting a chain reaction that eventually consumes entire brain regions. In the past 10 years scientists have learned that such a process may be at work not only in mad cow and other exotic diseases but also in major neurodegenerative disorders, including Alzheimer's, Parkinson's, amyotrophic lateral sclerosis (also known as ALS or Lou Gehrig's disease) and the concussion-related dementia of football players and boxers.
This article was originally published with the title Seeds of Dementia.