Gmitterová K, Heinemann U, Bodemer M, Krasnianski A, Meissner B, Kretzschmar H A, Zerr I
National Reference Centre for Transmissible Spongiform Encephalopathies, Department of Neurology, Georg-August University Goettingen, Germany.
Neurobiol Aging. 2009 Nov;30(11):1842-50. doi: 10.1016/j.neurobiolaging.2008.01.007. Epub 2008 Mar 6.
The 14-3-3 protein is a physiological cellular protein expressed in various tissues, and its release to CSF reflects extensive neuronal damage as in Creutzfeldt-Jakob disease (CJD), but also in other neurological diseases. 14-3-3 protein in CSF in the proper clinical context is a reliable diagnostic tool for sporadic CJD. However, the sensitivity varies across molecular CJD subtypes.
We determined the level of the 14-3-3 protein in CSF from 70 sporadic CJD patients with distinct molecular subtypes using an improved enzyme-linked immunosorbent assay (ELISA) protocol technique.
The 14-3-3 levels varied markedly across various molecular subtypes. The most elevated levels of 14-3-3 protein were observed in the frequently occurring and classical subtypes, whereas the levels were significantly lower in the subtypes with long disease duration and atypical clinical presentation. PRNP codon 129 genotype, PrP(sc) isotype, disease stage and clinical subtype influenced the 14-3-3 level and the test sensitivity.
The 14-3-3 protein levels differ across molecular subtypes and might be used for their early pre-mortem identification when the codon 129 genotype is known, especially for the less common molecular subtypes such as MV2 and MM2.
14-3-3蛋白是一种在多种组织中表达的生理性细胞蛋白，其释放到脑脊液中反映了广泛的神经元损伤，如在克雅氏病（CJD）中，但在其他神经疾病中也存在。在适当的临床背景下，脑脊液中的14-3-3蛋白是散发性CJD的可靠诊断工具。然而，其敏感性在不同分子型CJD亚型中有所不同。
我们使用改进的酶联免疫吸附测定（ELISA）方案技术，测定了70例具有不同分子亚型的散发性CJD患者脑脊液中14-3-3蛋白的水平。
14-3-3水平在不同分子亚型中差异显著。在常见和经典亚型中观察到14-3-3蛋白水平最高，而在病程长和临床表现不典型的亚型中水平显著较低。PRNP密码子129基因型、PrP(sc)亚型、疾病阶段和临床亚型影响14-3-3水平和检测敏感性。
14-3-3蛋白水平在不同分子亚型中存在差异，当已知密码子129基因型时，尤其是对于MV2和MM2等不太常见的分子亚型，可用于其生前早期识别。
