fishing for prion protein function钓朊蛋白的功能.pdfVIP

Primer Fishing for Prion Protein Function Roberto Chiesa*, David A. Harris* he prion protein (PrP) is a membrane-anchored, Although PrP knockout mice display no major anatomical neuronal glycoprotein whose normal function is or developmental defects, a bewildering variety of subtle Tuncertain, but which plays a crucial role in prion abnormalities have been described in these mice (reviewed diseases, a class of fatal neurodegenerative disorders of in [12]). These include altered circadian rhythms [13] humans and animals [1]. Bovine spongiform encephalopathy and olfaction [14], abnormalities in neuronal transmission (“mad cow disease”) and kuru, which are transmitted by and electrical activity [15], defective proliferation eating contaminated tissues, are the best known examples of and differentiation of neural precursor cells [16] and these disorders, which also occur in inherited and sporadic hematopoietic stem cells [17], increased sensitivity to forms. In prion diseases, the normal, endogenous form of hypoxia, ischemia, and seizures [18], and enhanced PrP (PrPC) undergoes transformation to a conformationally resistance to microbial infections [19]. Although intriguing, altered version (PrPSc) that accumulates in the brain none of these reported abnormalities has provided a as sticky, insoluble aggregates. This process leads to definitive clue to the normal function of PrPC. neuronal dysfunction and progressive neurodegeneration, Studies on the cell biology of PrPC have also failed to for which there is no effective treatment. Unlike other provide