Newswise — CLEVELAND — The National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University will receive up to $20 million in a renewed grant from the Centers for Disease Control and Prevention (CDC) to better understand the hows and whys of prion diseases – degenerative brain conditions found in both humans and animals – are evolving and spreading.
The five-year grant period began this month and will allow the center to continue 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, damaging nerve cells. The damage results in small spongy holes in the brain tissue.
There is no known cure for these disorders, which rapidly hasten death.
The first of its kind in the US
The NPDPSC is the only center of its kind in the U.S. and coordinates autopsies and neuropathology examinations of suspected prion disease cases from across the country, said Brian Appleby, director of the NPDPSC and the center’s principal investigator. Local autopsies are performed on site, and other cases are performed elsewhere through a network of providers coordinated by the NPDPSC. The Surveillance Center was originally established in 1997 at Case Western Reserve University’s Division of Neuropathology by Pierluigi Gambetti.
“The NPDPSC collects tissue samples and clinical information from prion disease cases to track its occurrence and investigate possible cases where these diseases have been acquired from other people or animals, such as chronic wasting disease transmitted from deer to humans. The additional grant money will allow us to continue surveillance and monitoring for emerging zoonotic prion diseases,” said Appleby.
“The center also operates a national clinical reference laboratory for prion disease and performs cerebrospinal fluid testing. CDC funding is key to setting up brain tissue testing and the use of a relatively new diagnostic test called RT-QuIC (real time quaking induced conversion), which has revolutionized the way the disease is clinically diagnosed. We are pleased to receive this grant renewal to continue this important research.”
Prion diseases unlike other brain conditions
Human prion diseases include Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia. Animal prion diseases consist of bovine spongiform encephalopathy, commonly known as “mad cow disease”, chronic wasting disease in deer, and scrapie in sheep and goats.
Symptoms of prion diseases in humans include memory loss, difficulty speaking and walking, and behavioral changes—symptoms that closely resemble 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 by mutations in the PRNP gene that encodes prion proteins, or sporadically when prion proteins fold abnormally for an unknown 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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