不同遗传型朊病毒病中国患者脑脊液样本中14-3-3蛋白和总tau蛋白的分析
Profiles of 14-3-3 and Total Tau in CSF Samples of Chinese Patients of Different Genetic Prion Diseases.
作者信息
Chen Cao, Hu Chao, Shi Qi, Zhou Wei, Xiao Kang, Wang Yuan, Liu Lian, Chen Jia, Xia Ying, Dong Xiao-Ping
机构信息
State Key Laboratory of Infectious Disease Prevention and Control, NHC Key Laboratory of Medical Virology and Viral Diseases, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China.
Center for Biosafety Mega-Science, Chinese Academy of Sciences, Wuhan, China.
出版信息
Front Neurosci. 2019 Sep 4;13:934. doi: 10.3389/fnins.2019.00934. eCollection 2019.
BACKGROUND
The abnormal alterations of proteins 14-3-3 and tau in cerebrospinal fluid (CSF) are widely used for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), while the situations of CSF biomarkers in genetic prion diseases (gPrDs), particularly in Chinese gPrDs patients, have not been well documented.
METHODS
Here, with the help of commercial 14-3-3 and total tau ELISA kits, we evaluated the levels of proteins 14-3-3 and tau in the CSF samples of 140 Chinese patients of 14 different types of gPrDs.
RESULTS
We found that CSF 14-3-3 ELISA values in the patients with P102L GSS and D178N FFI were remarkably low, while those in the patients with T188K, E196A, and E200K gCJD were relatively high. Linear correlation assays identified a positive correlation between positive rate in Western blot (WB) and ELISA values of CSF 14-3-3. ELISA assays for total tau in CSF samples identified relatively high levels in the cases of T188K, E196A, and E200K gCJD (median: 133840.81, 159992.80, and 153342.92 AU/ml), but relatively low levels in those of P102L GSS and D178N FFI (median: 64397.77 and 43856.79 AU/ml).
CONCLUSION
These data illustrate heterogeneous profiles of CSF 14-3-3 and tau in various types of gPrDs, depending on the differences in the mutations in .
背景
脑脊液（CSF）中蛋白质14-3-3和tau的异常变化被广泛用于散发性克雅氏病（sCJD）的诊断，而遗传朊病毒病（gPrDs），特别是中国gPrDs患者的脑脊液生物标志物情况尚未得到充分记录。
方法
在此，借助商用14-3-3和总tau ELISA试剂盒，我们评估了140例14种不同类型gPrDs中国患者脑脊液样本中蛋白质14-3-3和tau的水平。
结果
我们发现，P102L GSS和D178N FFI患者的脑脊液14-3-3 ELISA值显著较低，而T188K、E196A和E200K gCJD患者的脑脊液14-3-3 ELISA值相对较高。线性相关性分析确定了蛋白质印迹法（WB）阳性率与脑脊液14-3-3 ELISA值之间存在正相关。脑脊液样本总tau的ELISA分析确定，T188K、E196A和E200K gCJD患者的总tau水平相对较高（中位数：133840.81、159992.80和153342.92 AU/ml），而P102L GSS和D178N FFI患者的总tau水平相对较低（中位数：64397.77和43856.79 AU/ml）。
结论
这些数据表明，不同类型gPrDs患者脑脊液14-3-3和tau的情况存在异质性，这取决于[此处原文缺失相关内容]中突变的差异。
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