Nielsen Janni, Lauritsen Johanne, Pedersen Jannik N, Nowak Jan S, Bendtsen Malthe K, Kleijwegt Giulia, Lusser Kaija, Pitarch Laia C, Moreno Julián V, Schneider Matthias M, Krainer Georg, Goksøyr Louise, Khalifé Paul, Kaalund Sanne Simone, Aznar Susana, Kjærgaard Magnus, Sereikaité Vita, Strømgaard Kristian, Knowles Tuomas P J, Nielsen Morten Agertoug, Sander Adam F, Romero-Ramos Marina, Otzen Daniel E
Interdisciplinary Nanoscience Center (iNANO), Aarhus University, Aarhus C, Denmark.
DANDRITE & Department of Biomedicine, Aarhus University, Aarhus C, Denmark.
NPJ Parkinsons Dis. 2024 Jul 29;10(1):139. doi: 10.1038/s41531-024-00747-6.
α-Synuclein (α-syn) accumulates as insoluble amyloid but also forms soluble α-syn oligomers (αSOs), thought to be even more cytotoxic than fibrils. To detect and block the unwanted activities of these αSOs, we have raised 30 monoclonal antibodies (mAbs) against different forms of αSOs, ranging from unmodified αSOs to species stabilized by lipid peroxidation products and polyphenols, αSOs formed by C-terminally truncated α-syn, and multivalent display of α-syn on capsid virus-like particles (cVLPs). While the mAbs generally show a preference for αSOs, they also bind fibrils, but to variable extents. Overall, we observe great diversity in the mAbs' relative affinities for monomers and αSOs, varied requirements for the C-terminal extension of α-syn, and only a modest effect on α-syn fibrillation. Several mAbs show several orders of magnitude preference for αSOs over monomers in in-solution studies, while the commercial antibody MJF14 only bound 10-fold more strongly to αSOs than monomeric α-syn. Gratifyingly, seven mAbs almost completely block αSO permeabilization of membrane vesicles. Five selected mAbs identified α-syn-related pathologies like Lewy bodies (LBs) and Lewy Neurites, as well as Glial Cytoplasmic Inclusions in postmortem brains from people diagnosed for PD, dementia with LBs or multiple system atrophy, although to different extents. Three mAbs were particularly useful for pathological evaluation of postmortem brain human tissue, including early stages of PD. Although there was no straightforward connection between the mAbs' biophysical and immunohistochemical properties, it is encouraging that this comprehensive collection of mAbs able to recognize different aggregated α-syn species in vitro also holds diagnostic potential.
α-突触核蛋白(α-syn)会以不溶性淀粉样蛋白的形式积聚,但也会形成可溶性α-突触核蛋白寡聚体(αSOs),人们认为后者的细胞毒性甚至比纤维原更甚。为了检测并阻断这些αSOs的有害活性,我们制备了30种针对不同形式αSOs的单克隆抗体(mAbs),这些αSOs的形式多样,从不修饰的αSOs到由脂质过氧化产物和多酚稳定的种类、由C端截短的α-突触核蛋白形成的αSOs,以及α-突触核蛋白在衣壳病毒样颗粒(cVLPs)上的多价展示形式。虽然这些mAbs通常对αSOs表现出偏好,但它们也会与纤维原结合,不过结合程度各不相同。总体而言,我们观察到mAbs对单体和αSOs的相对亲和力存在很大差异,对α-突触核蛋白C端延伸的要求各不相同,并且对α-突触核蛋白纤维化的影响较小。在溶液研究中,几种mAbs对αSOs的偏好比对单体高几个数量级,而商业抗体MJF14与αSOs的结合强度仅比单体α-突触核蛋白强10倍。令人欣慰的是,七种mAbs几乎完全阻断了膜囊泡的αSOs通透作用。五种选定的mAbs识别出了α-突触核蛋白相关的病理特征,如路易小体(LBs)和路易神经突,以及在被诊断患有帕金森病(PD)、路易小体痴呆或多系统萎缩的人的尸检大脑中的胶质细胞质包涵体,尽管程度不同。三种mAbs对尸检人脑组织的病理评估特别有用,包括PD的早期阶段。虽然mAbs的生物物理性质和免疫组织化学性质之间没有直接联系,但令人鼓舞的是,这一能够在体外识别不同聚集α-突触核蛋白种类的mAbs综合集合也具有诊断潜力。