Prion protein

The prion protein (PrP) is a protein that in humans is encoded by the PRNP gene. Prion proteins are best known for their role in prion diseases, a group of progressive neurodegenerative disorders that affect both humans and animals. These diseases are characterized by the accumulation of an abnormal form of the prion protein in the brain.

Structure

The prion protein is a glycoprotein that is predominantly expressed in the central nervous system, but it is also found in other tissues. The normal cellular form of the prion protein is referred to as PrP^C, while the disease-associated form is referred to as PrP^Sc. The conversion of PrP^C to PrP^Sc is a key event in the pathogenesis of prion diseases.

Function

The exact physiological function of the prion protein is not fully understood. However, it is believed to play a role in cellular signaling, cell adhesion, and the protection of cells from oxidative stress.

Prion Diseases

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), include Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and kuru in humans, as well as bovine spongiform encephalopathy (BSE) in cattle and scrapie in sheep.

Transmission

Prion diseases can be transmitted through infectious, genetic, or sporadic means. Infectious transmission occurs through exposure to contaminated tissues, while genetic forms are associated with mutations in the PRNP gene. Sporadic cases occur without any known risk factors or genetic mutations.

Research

Research into prion proteins and prion diseases is ongoing, with efforts focused on understanding the mechanisms of prion propagation, the development of diagnostic tools, and the search for effective treatments.

See also

• Protein folding
• Neurodegenerative disease
• Amyloid

External links

• PRNP gene information at NCBI
• Prion protein at UniProt

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