生物谷报道：Prion蛋白在神经细胞中的作用非常复杂，传统的认识它与疯牛病的发生有关，近来研究显示它与学习和记忆，神经系统其它疾病的发生和形成都有密切关系，加深了人们对它的认识和了解。本周Science报道，它是决定神经细胞凋亡的关键因子，这一项研究是基于体内研究结果，具有更强的说服力。由此可见，对一个蛋白的功能的认识，远不是过去认为的一个蛋白是一种功能，或执行一类功能，而有可能执行不同类的功能，为今后功能蛋白质组学研究提供良好的启示。
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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