Pathogenesis

It is called PrPSc (for Prion Protein Scrapie). Healthy cells have PrP-C (cellular) which has the same 253 amino acids.

The bends and twists in the chain of amino acids are different. However, the abnormal protein is produced in nerve cells and causes empty spaces or vacuoles which eventually leads to microscopically visible brain holes called spongiform degeneration. These particles are not viruses but they do multiply. Is the normal necessary protein PrP-C converted into a malignant lethal molecule by contact with the prion? Are some so-called “inherited diseases” really caused by prions? These diseases have been transmitted by brain operations or by corneal transplants from infected donors.

Recently prion-protein (PrP-C) deprived mice have been shown to have an abnormal cellular docking site for gamma aminobutyric acid (GABA), one of the brain’s chemical messengers, the result is an electrical abnormality with involuntary jerks and, in some cases, seizures. This may be connected with lack of synapses (cellular connections ) without which most nerve cells die. These infectious proteins which so closely resemble normal intracellular proteins.

Destruction of the protein part leads to loss of infectivity.