Fatal Protein

This text presents a clear and authoritative account of the prion diseases for the non-specialist. Prion diseases, which include Creutzfeld Jakob disease (CJD), BSE, scrapie, and kuru, are caused by a unique pathological process thought to involve a mutant, or 'prion' protein. Prion diseases can affect both humans and animals, and are inevitably fatal since they cause a neurodegenerative process in which the brain cells fall apart. No vaccines, preventative medicines, or treatments are available to halt or slow the inevitable progression of the disease. This study deals with the history of scrapie in sheep, the spread of kuru through cannibalism in Papua New Guinea, and BSE, which has claimed 170000 cattle in the United Kingdom and which may threaten the rest of Europe. There is a detailed description of the human prion diseases, and the evidence that the new variant form of CJD results from eating meat infected by BSE is explored. At the heart of the story is a discussion of the still controversial idea that the infectious agent in prion diseases is a normal brain protein which has taken on an abnormal, or mutant, shape (the "prion theory").