UK Dementia Research Institute, University College London, London, W1T 7NF, UK.
Institute of Aerospace Medicine, German Aerospace Center (DLR), 51147, Cologne, Germany.
EMBO Mol Med. 2024 Jul;16(7):1657-1674. doi: 10.1038/s44321-024-00083-5. Epub 2024 Jun 5.
Synucleinopathies such as Parkinson's disease (PD) are defined by the accumulation and aggregation of the α-synuclein protein in neurons, glia and other tissues. We have previously shown that destabilization of α-synuclein tetramers is associated with familial PD due to SNCA mutations and demonstrated brain-region specific alterations of α-synuclein multimers in sporadic PD patients following the classical Braak spreading theory. In this study, we assessed relative levels of disordered and higher-ordered multimeric forms of cytosolic α-synuclein in blood from familial PD with G51D mutations and sporadic PD patients. We used an adapted in vitro-cross-linking protocol for human EDTA-whole blood. The relative levels of higher-ordered α-synuclein tetramers were diminished in blood from familial PD and sporadic PD patients compared to controls. Interestingly, the relative amount of α-synuclein tetramers was already decreased in asymptomatic G51D carriers, supporting the hypothesis that α-synuclein multimer destabilization precedes the development of clinical PD. Our data, therefore suggest that measuring α-synuclein tetramers in blood may have potential as a facile biomarker assay for early detection and quantitative tracking of PD progression.
突触核蛋白病,如帕金森病(PD),其特征是α-突触核蛋白在神经元、神经胶质和其他组织中的积累和聚集。我们之前已经表明,由于 SNCA 突变导致的α-突触核蛋白四聚体的不稳定性与家族性 PD 有关,并根据经典的 Braak 传播理论,在散发性 PD 患者中证明了脑区特异性的α-突触核蛋白多聚体改变。在这项研究中,我们评估了具有 G51D 突变的家族性 PD 和散发性 PD 患者血液中细胞溶质α-突触核蛋白的无序和高级多聚体形式的相对水平。我们使用了一种适用于人类 EDTA-全血的体外交联方案。与对照组相比,家族性 PD 和散发性 PD 患者血液中的高级α-突触核蛋白四聚体的相对水平降低。有趣的是,无症状 G51D 携带者的α-突触核蛋白四聚体的相对数量已经减少,这支持了α-突触核蛋白多聚体不稳定先于临床 PD 发展的假说。因此,我们的数据表明,测量血液中的α-突触核蛋白四聚体可能具有作为早期检测和定量跟踪 PD 进展的简便生物标志物检测的潜力。