del Río José A, Gavín Rosalina
a Molecular and Cellular Neurobiotechnology, Institute for Bioengineering of Catalonia (IBEC) , Parc Científic de Barcelona, Barcelona , Spain.
b Department of Cell Biology, Physiology and Inmunology , Facultat de Biologia, Universitat de Barcelona , Barcelona , Spain.
Prion. 2016;10(1):25-40. doi: 10.1080/19336896.2015.1126038.
Since its discovery the cellular prion protein (encoded by the Prnp gene) has been associated with a large number of functions. The proposed functions rank from basic cellular processes such as cell cycle and survival to neural functions such as behavior and neuroprotection, following a pattern similar to that of Moore's law for electronics. In addition, particular interest is increasing in the participation of Prnp in neurodegeneration. However, in recent years a redefinition of these functions has begun, since examples of previously attributed functions were increasingly re-associated with other proteins. Most of these functions are linked to so-called "Prnp-flanking genes" that are close to the genomic locus of Prnp and which are present in the genome of some Prnp mouse models. In addition, their role in neuroprotection against convulsive insults has been confirmed in recent studies. Lastly, in recent years a large number of models indicating the participation of different domains of the protein in apoptosis have been uncovered. However, after more than 10 years of molecular dissection our view is that the simplest mechanistic model in PrP(C)-mediated cell death should be considered, as Ockham's razor theory suggested.
自从细胞朊蛋白（由Prnp基因编码）被发现以来，它已与大量功能相关联。所提出的功能范围从基本的细胞过程如细胞周期和存活到神经功能如行为和神经保护，遵循类似于电子学中摩尔定律的模式。此外，Prnp在神经退行性变中的参与越来越受到特别关注。然而，近年来这些功能开始重新定义，因为先前归因的功能实例越来越多地与其他蛋白质重新关联。这些功能大多与所谓的“Prnp侧翼基因”相关，这些基因靠近Prnp的基因组位点，并且存在于一些Prnp小鼠模型的基因组中。此外，它们在抗惊厥性损伤的神经保护中的作用在最近的研究中得到了证实。最后，近年来发现了大量表明该蛋白不同结构域参与细胞凋亡的模型。然而，经过十多年的分子剖析，我们的观点是，正如奥卡姆剃刀理论所建议的，应考虑PrP(C)介导的细胞死亡中最简单的机制模型。
