The pathogenesis of prion disease is due to misfolding of the cellular prion protein(PrP~C)into a pathogenic isoform(PrP~(Sc)).Although biological functions of PrPC are still unclear,it is assumed that PrP~C plays an important role in copper metabolism,lipoid absorption and cell signal mediation.In addition,PrP~C can interact with caveolin-1 to activate Fyn and act as a receptor to activate cAMP/PKA through interacting with PrP~C-bound peptides and cellular signaling pathways by changing the level of intracellular calcium.%朊病毒病的发生是由于细胞正常朊蛋白PrP~C转变成了异常构象的PrP~(Sc)形式.PrP~C 的生理学功能目前尚不完全明确,可能与铜离子代谢、脂质摄取以及细胞信号传递有关.PrP~C可以与小窝蛋白相互作用而活化Fyn非受体酪氨酸激酶从而引起下游信号通路的转导;可以作为受体与PrP~C键合多肽结合后激活cAMP/PKA信号通路;以及引起细胞内钙离子浓度变化而活化信号通路.
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