the expanding universe of prion diseases朊病毒疾病的宇宙膨胀.pdfVIP

Review The Expanding Universe of Prion Diseases Joel C. Watts, Aru Balachandran, David Westaway* ABSTRACT 2 and Chromosome 20 in mice and humans, respectively [2]. ABSTRACT The epidemiological appearance of certain human prion rions cause fatal and transmissible neurodegenerative diseases in genetic and sporadic forms (not the case with disease. These etiological infectious agents are formed other classes of infectious pathogens) is also compatible with Pin greater part from a misfolded cell-surface protein the concept of aberrant metabolism of a ‘‘host’’ cellular called PrPC. Several mammalian species are affected by the component. Indeed, a tri-modal epidemiological diseases, and in the case of ‘‘mad cow disease’’ (BSE) the agent manifestation is a unique feature of prion diseases and has a tropism for humans, with negative consequences for unique in the realm of medical biology, blurring the dividing agribusiness and public health. Unfortunately, the known lines between metabolic and infectious diseases. In the case of universe of prion diseases is expanding. At least four novel inherited prion diseases such as familial CJD (now referred to prion diseases—including human diseases variant as genetic CJD), vertical transmission is not by an infectious Creutzfeldt-Jakob disease (vCJD) and sporadic fatal insomnia route but by germ-line inheritance of a mutated form of the (sFI), bovine amyloidotic spongiform encephalopathy (BASE), prion protein. Here the mutations may lead to misfolding by and Nor98 of sheep—have been identified in the last ten enco