Pei Yu, Maitta Robert W
University Hospitals Cleveland Medical Center, Cleveland, OH, United States.
Case Western Reserve University School of Medicine, Cleveland, OH, United States.
Heliyon. 2019 Oct 18;5(10):e02590. doi: 10.1016/j.heliyon.2019.e02590. eCollection 2019 Oct.
Parkinson's disease (PD) is the second most common neurodegenerative condition and intracellular deposition of Lewy bodies in the substantia nigra (SN), which can cause dopaminergic neuronal death, is the hallmark of this syndrome. α-synuclein (syn) is a small protein expressed mainly in neurons but can also be found in a number of tissues. It can be present as a soluble monomer under normal physiological conditions, but can be toxic in its oligomeric or fibrillary forms. Most of the available literature has focused on the effects of α-syn pathology in the mechanisms leading to PD. However, the normal functions of α-syn still remain to be fully elucidated. Notably, α-syn in the hematopoietic system seems to mediate important functions as indicated by anemia and incomplete cell maturation when this protein is absent. This review will summarize basic genetic and structural findings, and critical information that suggests an essential role of α-syn in the development and activation of the hematopoietic system and immunity.
帕金森病(PD)是第二常见的神经退行性疾病,黑质(SN)中路易小体的细胞内沉积会导致多巴胺能神经元死亡,这是该综合征的标志。α-突触核蛋白(α-syn)是一种主要在神经元中表达的小蛋白,但也可在许多组织中发现。在正常生理条件下,它可以以可溶性单体的形式存在,但在其寡聚体或纤维状形式下可能具有毒性。大多数现有文献都集中在α-突触核蛋白病理学在导致帕金森病的机制中的作用。然而,α-突触核蛋白的正常功能仍有待充分阐明。值得注意的是,当这种蛋白缺失时,造血系统中的α-突触核蛋白似乎介导重要功能,如贫血和细胞成熟不完全所示。本综述将总结基本的遗传和结构发现,以及表明α-突触核蛋白在造血系统发育和激活以及免疫中起重要作用的关键信息。