朊蛋白相关蛋白Shadoo与朊病毒病

朊病毒病，即传染性海绵状脑病（transmissible spongiform encephalopathies, TSEs），是一类传染性、致死性神经退行性疾病。在朊病毒病的病理过程中，细胞正常朊蛋白PrP。转化为异常构象的PrP是至关重要的，但是PrP‘的正常生理功能仍不清楚。国外学者利用比较基因组学发现了-个新的朊蛋白相关蛋白-shadoo（Sho）。Sho与PrP。在氨基酸序列和细胞定位的相似性及主要在脑组织表达，使它成为-个非常值得研究的PrP相关蛋白。对Sho可能存在的与PrP。重叠的功能甚至直接相互作用的研究工作，将对今后揭示PrPc正常生理功能以及揭示Pfion病发病机制具有重要现实意义。

Prion-Like Protein Shadoo and Prion Disease

Prion disease, also known as transmissible spongiform encephalopathies （TSEs）, is a kind of infectious, fatal neurodegenerative diseases. In pathogenesis of Prion disease, confor- mational change of the cellular prion protein PrPc into disease-related conformer termed PrPsc is a crucial step. However, the normal physiological function of PrPc remains unclear. Based on compar- ative genomics, foreign researchers have discovered a new prion-related protein： Shadoo （Sho） ~ Striking sequence similarity of Sho to PrPc and its specific expression in brain allow the hypothesis that Sho might be functionally related to PrP and important for understanding prion disease. This re- view focuses on the relationship between Sho and prion diseases. The possible overlapping functions or even direct interaction between Sho and PrPc might help to reveal normal function of PrPc as well as the mechanism underlying Prion disease.