Prion Diseases I. The Etiology of Prion Diseases

(Mansour bin Faris Hussein and Saud bin August)

---

Abstract

Prion diseases or transmissible spongiform encephalopathies (TSE’s) constitute an unusual group of progressive neurodegenerative disorders of man and animals characterized clinically by dementia, neurologic and motor disturbances, and ultimate fatality, and pathologically by neuronal vacuolations, gliosis and astrocytosis in the brain tissue. These diseases are peculiar in that they are both infectious and inherited, and are the only known diseases believed to be caused by an “infectious” protein. That protein is a protease-resistant, insoluble prion protein capable of replicating in neurons in the absence of nucleic acid; it is referred to as the “prion protein of scrapie,” or PrPSC , and is believed to arise as a result of misfolding of its normal counterpart in the cells, PrPC.
PDF

References