RT-QuIC: a new test for sporadic CJD.

RT-QuIC: a new test for sporadic CJD.
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DOI:
10.1136/practneurol-2018-001935
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发表时间:
2019-03
影响因子:
2.8
通讯作者:
Green AJE
Green AJE
中科院分区:
其他
文献类型:
--
作者:
Green AJE

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散发性克雅氏病(CJD)的诊断可能很困难,但实时振荡诱导转换(RT-QuIC)检测对其临床诊断产生了相当大的影响。该技术利用了在脑脊液(CSF)中发现的朊病毒蛋白(PrPSc)的错误折叠病理形式诱导正常PrP转化为错误折叠形式,随后聚集的能力。使用荧光染料在真实的时间监测这些错误折叠的PrP聚集体的形成。目前,英国国家CJD研究与监测单位进行的CSF RT-QuIC的灵敏度为92%,特异性为100%。RT-QuIC迹线的判读受到CSF红细胞和白色细胞计数升高以及总蛋白浓度升高的影响。我们建议用于RT-QuIC分析的CSF样本应澄清无色,白色细胞计数<10 x10^6/L,总蛋白浓度<1 g/L。
The diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) can be difficult, but the real-time quaking-induced conversion (RT-QuIC) assays have made a considerable impact on its clinical diagnosis. This technique exploits the ability of the misfolded pathological form of prion protein (PrPSc) found in cerebrospinal fluid (CSF) to induce conversion of normal PrP to the misfolded form, which subsequently aggregates. The formation of these aggregates of misfolded PrP is monitored in real time using fluorescent dyes. The current sensitivity of CSF RT-QuIC undertaken at the UK National CJD Research & Surveillance Unit is 92% and the specificity is 100%. The interpretation of the RT-QuIC traces is affected by the presence of raised CSF red and white cells counts and elevated total protein concentrations. We recommend that CSF samples for RT-QuIC analysis are clear and colourless with a white cell count of <10 x10^6/L and have a total protein concentration of <1 g/L.
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