Prion diseases

April 11, 2016

Article: Prion diseases

In this article, Dr. Raymond Roos of the University of Chicago reviews the group of diseases known as prion diseases, also referred to as the subacute spongiform encephalopathies. These diseases have a subacute to chronic clinical course with a similar neuropathology. All the diseases are transmissible and induced by an abnormal misfolded form of the prion protein that is extremely resistant to physical and chemical inactivation. The unusual nature of the transmissible agent and the emergence of variant Creutzfeldt-Jakob disease (as a result of ingestion of contaminated beef) have had a significant impact on public health in addition to science and medicine. New diagnostic tests, such as protein misfolding cyclic amplification and real time quaking-induced conversion, and new ideas about treatment of the subacute spongiform encephalopathies are discussed..

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