Prion Diseases : A Review : II. Prion Diseases in Man and Animals

(Mansour Faris Hussein and Saud bin APRA)

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Abstract

o date, a total of 13 prion diseases have been recognized in man and animals. The human diseases are: Kuru, Creutzfeldt-Jakob disease (CJD), variant CJD, Gertmann-Straussler-Scheinker Syndrome, fatal familial insomnia and Alpers8217; disease. The animal diseases are: scrapie, transmissible mink encephalopathy, chronic wasting disease, bovine spongiform encephalopathy, feline spongiform encephalopathy, wild ungulates encephalopathy and spongiform encephalopathy of the ostrich. Early diagnosis and treatment of these diseases have bewildered the scientific community. The fact that the prion protein (PrP) is derived from the host 8211; given that PrP is the sole cause of disease - makes it almost impossible to develop direct serologic tests and vaccines for the diagnosis and prevention of these diseases. At present, diagnosis is largely based on clinical and post-mortem findings, detection of abnormal prion protein by immuno-cytochemistry, Western blot, infra-red spectroscopy and biochemical examination of cerebraospinal fluid and blood of suspected patients. Methods are currently being evaluated for the identification of 8220;capture8221; agents that specifically bind to misfolded prion protein (PrPSC), and for amplification of the interconversion of normal prion protein into PrPSC. No treatment is available yet for prion diseases, but several candidate drugs are being investigated that could prevent PrPSC formation, interfere with its conversion and/or metabolism or reverse it into the normally folded, harmless form.
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