Qin K, O'Donnell M, Zhao R Y
Department of Pathology, University of Maryland School of Medicine, Baltimore, MD 21201, USA.
Neuroscience. 2006 Aug 11;141(1):1-8. doi: 10.1016/j.neuroscience.2006.04.057. Epub 2006 Jun 15.
Conversion of normal cellular prion protein to the diseased form plays an essential role in transmissible spongiform encephalopathies such as mad cow disease and Creutzfeldt-Jakob disease. However, the normal physiological function of prion protein remains elusive. Doppel, a German synonym of double, was initially identified as a prion-like protein due to its structural and biochemical similarities. However, emerging evidence suggests that function of prion protein is more antagonistic to Doppel than synergistic. In this review, basic biochemical and structural similarities of prion protein and Doppel are introduced; evidence demonstrating antagonistic interaction of prion protein with Doppel is presented; and a potential novel activity of Doppel and prion protein in spermatogenesis, which could stimulate new avenues for research, is discussed.
正常细胞朊病毒蛋白向致病形式的转化在诸如疯牛病和克雅氏病等传染性海绵状脑病中起着至关重要的作用。然而，朊病毒蛋白的正常生理功能仍然不清楚。Doppel是德语中“双”的同义词，由于其结构和生化相似性，最初被鉴定为一种朊病毒样蛋白。然而，新出现的证据表明，朊病毒蛋白的功能与其说是与Doppel协同，不如说是与其拮抗。在这篇综述中，介绍了朊病毒蛋白和Doppel的基本生化和结构相似性；展示了证明朊病毒蛋白与Doppel存在拮抗相互作用的证据；并讨论了Doppel和朊病毒蛋白在精子发生中的一种潜在新活性，这可能会为研究开辟新途径。
