生物谷報道：Prion蛋白在神經(jīng)細胞中的作用非常復雜,，傳統(tǒng)的認識它與瘋牛病的發(fā)生有關(guān),，近來研究顯示它與學習和記憶，神經(jīng)系統(tǒng)其它疾病的發(fā)生和形成都有密切關(guān)系,，加深了人們對它的認識和了解,。本周Science報道，它是決定神經(jīng)細胞凋亡的關(guān)鍵因子,，這一項研究是基于體內(nèi)研究結(jié)果,，具有更強的說服力。由此可見,，對一個蛋白的功能的認識,，遠不是過去認為的一個蛋白是一種功能,，或執(zhí)行一類功能，而有可能執(zhí)行不同類的功能,，為今后功能蛋白質(zhì)組學研究提供良好的啟示,。

Cross-Linking Cellular Prion Protein Triggers Neuronal Apoptosis in Vivo

Laura Solforosi 1, Jose R. Criado 2, Dorian B. McGavern 2, Sebastian Wirz 3, Manuel Sánchez-Alavez 2, Shuei Sugama 3, Lorraine A. DeGiorgio 4, Bruce T. Volpe 4, Erika Wiseman 1, Gil Abalos 1, Eliezer Masliah 5, Donald Gilden 6, Michael B. Oldstone 2, Bruno Conti 3, R. Anthony Williamson 1*

Neuronal death is a prominent, but poorly understood pathological hallmark of prion disease. Significantly, in the absence of the cellular prion protein (PrPC), the disease-associated isoform, PrPSc, appears not to be intrinsically neurotoxic, suggesting that PrPC itself may participate directly in the prion neurodegenerative cascade. Here, cross-linking PrPC in vivo using specific monoclonal antibodies was found to trigger rapid and extensive apoptosis in hippocampal and cerebellar neurons. These findings suggest that PrPC functions in the control of neuronal survival and provides a model to explore whether cross-linking of PrPC by oligomeric PrPSc can promote neuronal loss during prion infection.

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