MacDonald S T, Sutherland K, Ironside J W
CJD Surveillance Unit, Western General Hospital, Edinburgh.
Neurodegeneration. 1996 Mar;5(1):87-94. doi: 10.1006/neur.1996.0012.
Creutzfeldt-Jakob disease (CJD) is the most common spongiform encephalopathy affecting humans. Prion protein (PrP) immunohistochemistry may be useful for studying the localization of prion protein and assessing its role in CJD, the accumulation of a specific protease resistant PrP isoform being apparently pathognomic to the spongiform encephalopathies. However, a number of factors influence the results of immunostaining, making interpretation and comparisons between the staining of different PrP antisera difficult. This study has examined qualitatively and quantitatively the staining produced by four antisera raised to a variety of prion protein homologues in two cases of CJD and two age-matched controls. Quantitative analysis was provided through the use of custom designed image analysis software. Kuru, granular and multicentric plaques, cellular, perivacuolar and white matter PrP deposits were observed in CJD cases with all four antisera. No significant immunostaining was seen in the control tissue. Some antibody specific staining patterns were observed qualitatively; however, quantitative analysis showed statistically significant correlations between all the antisera on the diseased brain tissue. Prion protein immunohistochemistry is thus useful in interpreting patterns of protein distribution in diseased brain but care may be required in interpreting the results of a single antibody.
克雅氏病（CJD）是最常见的影响人类的海绵状脑病。朊病毒蛋白（PrP）免疫组化可能有助于研究朊病毒蛋白的定位，并评估其在克雅氏病中的作用，一种特定的抗蛋白酶PrP异构体的积累显然是海绵状脑病的病理特征。然而，许多因素会影响免疫染色的结果，使得不同PrP抗血清染色之间的解释和比较变得困难。本研究对两例克雅氏病患者和两名年龄匹配的对照者，定性和定量地检测了四种针对多种朊病毒蛋白同源物产生的抗血清所产生的染色情况。通过使用定制设计的图像分析软件进行定量分析。在两例克雅氏病患者中，使用所有四种抗血清均观察到库鲁病、颗粒状和多中心斑块、细胞、空泡周围和白质PrP沉积。在对照组织中未观察到明显的免疫染色。定性观察到了一些抗体特异性染色模式；然而，定量分析表明，所有抗血清在患病脑组织上的染色之间存在统计学上的显著相关性。因此，朊病毒蛋白免疫组化有助于解释患病大脑中蛋白质的分布模式，但在解释单一抗体的结果时可能需要谨慎。
