神经元的死亡被认为是朊病的特点,这是由于细胞表面的唾液酸糖蛋白由正常的含有大量α螺旋的结构变成了异常的β折叠丰富的、具有蛋白酶抗性的结构。最近的研究结果表明,朊病的复制可以被抗PrP的单克隆抗体所抑制,能延长朊病毒的潜伏期,这增加了人们用抗体来治疗朊病的信心。
Neuronal cell death is considered to be a hallmark in prion diseases. These disorders are believed to result from the post-translational conversionof a normal cell membrane sialoglycoprotein, composed primarily of helical structure, into a disease specific isoform, that is rich in sheet and partially proteinase-resistant. Recent in vivo studies indicate that prion replication can be inhibited by anti-PrP monoclonal antibodies that led to the indefinite delay in the development of prion disease. Furthermore, these reports increased momentum about the use of antibody-based therapy in prion diseases.