Prion Protein

Prion Protein

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About PRN / Prion Protein:

Prion (PRN) proteins are defined as misfolded proteins that are able to transmit their shape upon normal proteins. Why this happens is currently not known, but they are considered the main characteristic in a range of fatal and sometimes transmissible neurodegenerative diseases.
For instance, prion isoforms of the prion protein have been hypothesized as having an integral cause in the development of mad cow disease, chronic wasting disease in deer, and scrapie in sheep. All of these prion diseases have no effective treatment as of yet, and unfortunately, they always lead to fatal consequences.

PRN Structure
Prions are known to form unfamiliar aggregates of proteins which have been associated with both cell death and tissue damage. The proteins that prions develop from is found throughout the body in both human and animal populations. However, in infectious form, the PrPSc curates a different structure and becomes immune to the protein-processing enzymes.

Prion Protein Types
Types of prion disease can include CJD and its variant, fatal insomnia, and Kuru. Prions can form amyloids, for aforementioned aggregates of proteins, and are linked to certain neurodegenerative diseases in old age, like Alzheimer’s and Parkinson’s.

PRN Function
The normal functioning of the Prion Protein and its physiological function is not entirely understood. It has been found that the activation of myelin repair seems to be caused of Prion proteins in the peripheral nerves.
Those with risk factors of prion disease include those with a family history of it, eating meat infected with ‘mad cow disease’ and exposure to contaminated medical equipment.