Wihan Jeanette, Battis Kristina, Hoffmann Alana, Windener Farina, Himmler Marcus, Varghese Anish, Koller Aron, Karnatz Isabell, Schubert Dirk W, Zunke Friederike, Xiang Wei, Kuhlmann Tanja, Winkler Jürgen
Division of Molecular Neurology, University Hospital Erlangen, Friedrich-Alexander-University (FAU) Erlangen-Nürnberg, 91054, Erlangen, Germany.
Project Center for Stem Cell Process Engineering, Fraunhofer Institute for Biomedical Engineering (IBMT), 97070, Würzburg, Germany.
Acta Neuropathol. 2025 Sep 19;150(1):33. doi: 10.1007/s00401-025-02933-z.
Oligodendroglial alpha-synuclein (aSyn) deposits are a key feature in the atypical parkinsonian disorder, multiple system atrophy (MSA) linked to profound myelin loss and neurodegeneration while precise cellular and molecular mechanisms remain unclear. We generated human oligodendrocytes (hOLs) from induced pluripotent stem cells to investigate the impact of aSyn on oligodendroglial morphology, differentiation, and function. We observed an aSyn-induced myelinogenic dysfunction characterized by impaired oligodendroglial process outgrowth, altered cell shape, and increased perinuclear accumulation of the tubulin polymerization promoting protein TPPP/p25α. These changes were associated with a reduced capacity to ensheath axons and were linked to compromised actin remodeling machinery. Actin imbalances were confirmed in post-mortem putaminal tissue from MSA patients. Treatment with a rho-associated protein kinase inhibitor rescued oligodendroglial process formation and improved ensheathment in aSyn-expressing hOLs. Our work emphasizes the aSyn-mediated interference with actin dynamics as a key pathogenic mechanism in MSA, pointing toward a novel therapeutic target for improving myelin maintenance.
少突胶质细胞α-突触核蛋白(aSyn)沉积是不典型帕金森病——多系统萎缩(MSA)的关键特征,与严重的髓鞘脱失和神经变性有关,而确切的细胞和分子机制仍不清楚。我们从诱导多能干细胞生成人少突胶质细胞(hOLs),以研究aSyn对少突胶质细胞形态、分化和功能的影响。我们观察到aSyn诱导的髓鞘生成功能障碍,其特征为少突胶质细胞突起生长受损、细胞形状改变以及微管蛋白聚合促进蛋白TPPP/p25α的核周积累增加。这些变化与包裹轴突的能力降低有关,并与肌动蛋白重塑机制受损有关。在MSA患者的尸检壳核组织中证实了肌动蛋白失衡。用一种与rho相关的蛋白激酶抑制剂治疗可挽救表达aSyn的hOLs中的少突胶质细胞突起形成并改善包裹情况。我们的工作强调aSyn介导的对肌动蛋白动力学的干扰是MSA中的关键致病机制,为改善髓鞘维持指出了一个新的治疗靶点。