Developments in diagnosis for prion diseases.
Developments in diagnosis for prion diseases.
复制标题
朊病毒疾病诊断的进展。
作者：
J. Tateishi;T. Kitamoto
PrP是人和动物海绵状脑病的诊断标记物。免疫印迹是一种敏感的方法，但需要新鲜或冷冻的非固定材料。使用福尔马林固定的石蜡包埋材料的免疫组织化学现在也被认为是敏感的，可以与各种处理后的免疫印迹法相媲美，特别是在染色前对组织切片使用水解式高压灭菌法。该方法的优点是可用于常规病理标本或保存时间较长的标本。PrP的库鲁菌斑型沉积提示PrP基因异常，而突触型沉积提示散发性CJD或特殊的家族性CJD。因此，PrP基因异常与PrP沉积有关，并改变临床症状及其进展。PrP基因分析可以使用临床前、临床或尸检材料进行。
The protease resistant isoform of prion protein (PrP) is a diagnostic marker of spongiform encephalopathies in humans and animals. Immunoblotting is a sensitive method but requires either fresh or frozen, unfixed materials. Immunohistochemistry using formalin-fixed, paraffin-embedded materials is now also considered to be sensitive and comparable to immunoblotting after various treatments, especially using the hydrolytic autoclaving method on tissue sections before staining. The advantage of this method is that it can be applied to routine pathology materials or long preserved materials. The kuru plaque-type deposition of PrP suggests abnormalities of the PrP gene, while synaptic-type deposition suggests either sporadic CJD or particular familial CJD. PrP gene abnormalities are thus related to PrP deposition and modify clinical symptoms and their progression. A PrP gene analysis can be done using either preclinical, clinical or post-mortem materials.