What Is Prion Protein? ~ Domenico Pratico, MD, FCPP
- April 29, 2024
Prion protein (PrP) is a protein expressed in various organs and tissues throughout our body, but it is
found in great abundance in the brain on the outer surface of the nerve cells, and for this reason
also called PrP cellular or PrPC. Due its structural flexibility PrPC interacts with a wide range of
partners, structures and compounds and is believed to be involved in many physiological functions
for the brain such as neuroprotection and maintaining the homeostasis of a structure called myelin
(the outer membrane of the nerves).
It exists in two forms: the normal form, PrPC, which we just described and the misfolded form, which
is typically associated with what we call āprion diseasesā and is referred as PrPSc.
At times the proteins can assume a pathologic shape (misfolding) for unknown reasons, due to
genetic mutations, or by exposure to an already misfolded protein.
The resulting abnormal structure can cause the misfolding of other apparently normal prion
proteins, which can then trigger stress for the cell normal function and ultimately cell death.
Altered and misfolded prion proteins cause āPrion Diseasesā which are transmissible, fatal
neurodegenerative diseases in humans and animals.
Among them, the transmissible spongiform encephalopathies (TSEs) including scrapie in sheep,
bovine spongiform encephalopathy in cattle (mad cow disease), the CreutzfeldtāJakob disease and
fatal insomnia in humans.
Prion diseases in mammals affect the structure of the brain or other neural tissue; all are
progressive, have no known effective treatment, and are always fatal.
It is imperative that more research is performed on this under investigated area of
neurodegeneration.
This effort should shed light not only on the mechanisms responsible for the transformation of a
normal prion protein into a pathologic and toxic one but also on possible therapeutic target and
pharmacologic approaches against this fatal disease.
Domenico PraticĆ², MD, is the Scott Richards North Star Charitable Foundation Chair for Alzheimerās Research, Professor and Director of the Alzheimerās Center at Temple, and Professor of Pharmacology at the Lewis Katz School of Medicine at Temple University
You can find out more information on Dr. Domenico Pratico’s research papers here.
Follow Dr Domenico Pratico‘s lab website here: Pratico Lab
