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 “prion diseases” 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 “Prion Diseases” 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 Creutzfeldt–Jakob 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 Praticò, MD, holds the position of the Scott Richards North Star Charitable Foundation Chair for Alzheimer’s Research and serves as a Professor and the Director at the Alzheimer’s Center at Temple, as well as a Professor of Pharmacology at Lewis Katz School of Medicine at Temple University.
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