Center of Clinical Research, Experimental Surgery and Translational Research, Biomedical Research Foundation of the Academy of Athens, 11527 Athens, Greece.
Int J Mol Sci. 2021 May 8;22(9):4994. doi: 10.3390/ijms22094994.
Accumulation of the neuronal presynaptic protein alpha-synuclein within proteinaceous inclusions represents the key histophathological hallmark of a spectrum of neurodegenerative disorders, referred to by the umbrella term a-synucleinopathies. Even though alpha-synuclein is expressed predominantly in neurons, pathological aggregates of the protein are also found in the glial cells of the brain. In Parkinson's disease and dementia with Lewy bodies, alpha-synuclein accumulates mainly in neurons forming the Lewy bodies and Lewy neurites, whereas in multiple system atrophy, the protein aggregates mostly in the glial cytoplasmic inclusions within oligodendrocytes. In addition, astrogliosis and microgliosis are found in the synucleinopathy brains, whereas both astrocytes and microglia internalize alpha-synuclein and contribute to the spread of pathology. The mechanisms underlying the pathological accumulation of alpha-synuclein in glial cells that under physiological conditions express low to non-detectable levels of the protein are an area of intense research. Undoubtedly, the presence of aggregated alpha-synuclein can disrupt glial function in general and can contribute to neurodegeneration through numerous pathways. Herein, we summarize the current knowledge on the role of alpha-synuclein in both neurons and glia, highlighting the contribution of the neuron-glia connectome in the disease initiation and progression, which may represent potential therapeutic target for a-synucleinopathies.
在一系列神经退行性疾病中,神经元突触前蛋白α-突触核蛋白在蛋白包涵体内的积累代表了关键的组织病理学特征,这些疾病统称为α-突触核蛋白病。尽管α-突触核蛋白主要在神经元中表达,但该蛋白的病理性聚集体也存在于大脑的神经胶质细胞中。在帕金森病和路易体痴呆中,α-突触核蛋白主要在形成路易体和路易神经突的神经元中积累,而在多系统萎缩中,该蛋白主要在少突胶质细胞的神经胶质细胞质包涵体中聚集。此外,在突触核蛋白病脑中还发现了星形胶质细胞增生和小胶质细胞增生,而星形胶质细胞和小胶质细胞均内化α-突触核蛋白,并有助于病理的传播。在生理条件下表达低水平至无法检测到该蛋白的情况下,α-突触核蛋白在神经胶质细胞中病理性积累的机制是一个研究热点。毫无疑问,聚集的α-突触核蛋白的存在会破坏神经胶质细胞的功能,并通过多种途径导致神经退行性变。在此,我们总结了目前关于α-突触核蛋白在神经元和神经胶质细胞中的作用的知识,强调了神经元-神经胶质连接组在疾病起始和进展中的作用,这可能是治疗α-突触核蛋白病的潜在靶点。