摘要
克雅病(CJD)是一种由朊病毒侵袭人类中枢神经系统引起的传染性疾病,临床上主要表现为患者快速进行性痴呆。该病潜伏期长,病死率高。目前该病诊断金标准是活体脑组织检测,由于采样损伤和伦理问题在实际工作中极难开展。亟需找到一种可靠的实验室检测方法在发病早期对病例进行甄别,以便为非CJD患者寻求其他治疗方案,为确诊CJD患者采取对症治疗和医学防护。现在实验室检测主要集中在血液基因测序和脑脊液生物标志物(14-3-3蛋白、Tau蛋白、Aβ蛋白等)的检测。Tau蛋白是目前最准确的CJD单一标志物。实验检测方法方面实时震动诱导转化(RT-QuIC)和连续蛋白质错误折叠循环扩增(sPMCA)均能检出极低量的错误折叠蛋白聚合物,是目前最为可靠的早期人类阮蛋白疾病的实验室检测手段。但Tau蛋白等生物标志物的研究目前还未达成世界范围内共识,需要更多的数据支持。
The Creutzfeldt-Jakob disease(CJD)is a kind of infectious diseases that attacks human central nervous system by prion.The main clinical manifestation is rapid progressive dementia.CJD has long incubation and high mortality.At present,the gold standard of CJD diagnosis is brain tissue test,which is very difficult to carry out in practice because of brain injury and ethical issues.Therefore,it is necessary to find a reliable laboratory testing method to discriminate cases in the early stage,so as to seek other treatment options for non-patients and take symptomatic treatment and medical protection for confirmed patients.Nowadays,the laboratory tests mainly focus on the detection of blood gene sequencing and cerebrospinal fluid biomarkers(14-3-3 protein,Tau protein,Aβprotein).Tau protein is considered to be the most accurate single marker of CJD.In terms of experimental detection methods,Real Time Quaking Enduced Conversion(RT-QuIC)and senal Protein Misfolding Cyclic Amplification(sPMCA)are the most reliable laboratory detection method for early human protein diseases which could detect very low amounts of misfolded protein polymers.However,the research on Tau protein and other biomarkers has not reached a worldwide consensus,and further support data are needed.
作者
刘怡芳
吕杰
LIU Yi-fang;LYU Jie(Infectious Diseases Department,Infectious Diseases Control Room,Tianjin Center Jor Disease Control and Prevention,Tianjin,300011,China)
出处
《职业与健康》
CAS
2020年第5期697-700,共4页
Occupation and Health
基金
国家科技重大专项课题(2017ZX10103007-002)。
关键词
克雅病
脑脊液
检测
Creutzfeldt-Jakob disease
Cerebrospinal fluid
Detection
