National Prion Disease Pathology Surveillance Center to receive up to $20 million in CDC grant for continued research into fatal neurodegenerative prion diseases in the brain

Newswise — CLEVELAND– Case Western Reserve University’s National Prion Disease Pathology Surveillance Center (NPDPSC) will receive up to $20 million in funding as part of a grant extension from the Centers for Disease Control and Prevention (CDC) to better understand how and why Prion diseases – degenerative brain disorders found in both humans and animals – develop and spread.

The five-year grant period began this month and will enable the center to continue its research and diagnostic activities related to prion diseases.

Prion diseases are different from other communicable diseases caused by viruses or bacteria. They develop when normal prion proteins in the brain change shape and clump together, causing damage to nerve cells. The damage results in small spongy holes in brain tissue.

There is no known cure for these conditions, which rapidly accelerate death.

First of its kind in the US

The NPDPSC is the only center of its kind in the US, coordinating autopsies and neuropathology studies of suspected cases of prion disease from across the country, said Brian Appleby, NPDPSC director and principal investigator of the center. Local autopsies are performed on site and other cases are performed elsewhere through a network of providers that NPDPSC coordinates. The surveillance center was originally established in 1997 by Pierluigi Gambetti at Case Western Reserve University’s Department of Neuropathology.

“NPDPSC collects tissue samples and clinical information from cases of prion disease to monitor its incidence and investigate possible cases where these diseases have been acquired from other humans or animals, such as chronic wasting disease transmitted from deer to humans. The additional grant money will allow we continue to monitor and monitor the emergence of new zoonotic prion diseases,” said Appleby.

“The center also manages the National Clinical Reference Laboratory for Prion Diseases and performs cerebrospinal fluid testing. The CDC funding is key to establishing brain tissue testing and the use of the relatively new diagnostic test called RT-QuIC (real-time quaking-induced conversion), which has revolutionized the way the disease is treated clinically. diagnosed. We are delighted to receive this grant extension to continue this important research.”

Prion diseases as opposed to other brain disorders

Human prion diseases include Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia. Prion diseases in animals consist of bovine spongiform encephalopathy, commonly known as “mad cow disease”, chronic wasting disease found in deer, and scrapie found in sheep and goats.

Symptoms of prion disease in humans include memory loss, difficulty speaking and walking, and behavioral changes — manifestations very similar to Alzheimer’s disease and other dementias, making the condition difficult to diagnose.

Prion diseases in humans can be acquired through contaminated food or contact with infected medical equipment, inherited through mutations in the PRNP gene encoding prion proteins, or sporadically when prion proteins fold abnormally for no known reason.

The most common form of prion disease in humans, Creutzfeldt-Jakob disease, was responsible for more than 500 deaths in 2020, according to the CDC.


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