Prion-like proteins drive several diseases of aging

September 5, 2013

Two leading neurology researchers have proposed a theory that could unify scientists' thinking about several neurodegenerative diseases and suggest therapeutic strategies to combat them.

The theory and backing for it are described in Nature.

Mathias Jucker and Lary Walker outline the emerging concept that many of the associated with aging, such as Alzheimer's and Parkinson's, are caused by specific proteins that misfold and aggregate into harmful seeds.

These seeds behave very much like the pathogenic agents known as prions, which cause , in deer, scrapie in sheep, and Creutzfeldt-Jakob disease in humans.

Walker is research professor at Yerkes National Primate Research Center, Emory University. Jucker is head of the Department of Cellular Neurology at the Hertie Institute for Clinical Brain Research at the University of Tübingen and the German Center for Neurodegenerative Diseases.

Unlike prion diseases, which can be infectious, Alzheimer's, Parkinson's, and other can not be passed from person to person under normal circumstances. Once all of these diseases take hold in the brain, however, it is increasingly apparent that the clumps of misfolded proteins spread throughout the nervous system and disrupt its function.

The authors were the first to show that a protein that is involved in Alzheimer's disease—known as amyloid-beta—forms prion-like seeds that stimulate the aggregation of other amyloid-beta molecules in and in . Since then, a growing number of laboratories worldwide have discovered that proteins linked to other neurodegenerative disorders also share key features with prions.

Age-related neurodegenerative disorders remain stubbornly resistant to the discovery of effective treatments. Jucker and Walker propose that the concept of pathogenic protein seeding not only could focus research strategies for these seemingly unrelated diseases, but it also suggests that therapeutic approaches designed to thwart prion-like seeds early in the disease process could eventually delay or even prevent the diseases.

Explore further: Designer compounds inhibit prion infection

More information:

Related Stories

Designer compounds inhibit prion infection

July 20, 2012

(Medical Xpress) -- A team of University of Alberta researchers has identified a new class of compounds that inhibit the spread of malfunctioning proteins in the brain that cause lethal neurodegenerative diseases in humans ...

New models advance the study of deadly human prion diseases

August 19, 2013

By directly manipulating a portion of the prion protein-coding gene, Whitehead Institute researchers have created mouse models of two neurodegenerative diseases that are fatal in humans. The highly accurate reproduction of ...

Recommended for you

Functional human liver cells grown in the lab

November 26, 2015

In new research appearing in the prestigious journal Nature Biotechnology, an international research team led by The Hebrew University of Jerusalem describes a new technique for growing human hepatocytes in the laboratory. ...


Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.