Kim Hongwon, Kang Soi, Cho Byounggook, An Saemin, Kim Yunkyung, Kim Jongpil
Department of Chemistry, Dongguk University, Pudong 1-gil 30, Jung-gu, Seoul, 04620, Republic of Korea.
Department of Chemistry and Chemical Biology, Rutgers, The State University of New Jersey, Piscataway, NJ, 08854, USA.
Adv Sci (Weinh). 2025 Apr;12(14):e2412548. doi: 10.1002/advs.202412548. Epub 2025 Feb 18.
Parkinson's disease (PD) is characterized by the progressive loss of dopaminergic neurons and the accumulation of α-synuclein aggregates, yet current models inadequately mimic the complex human brain environment. Recent advances in brain organoid models offer a more physiologically relevant platform for studying PD, however, iPSC-derived brain organoids require long maturation times and may not accurately represent the aged brain's epigenetics and cellular contexts, limiting their applicability for modeling late-onset diseases like PD. In this study, a novel approach for generating 3D-induced dopaminergic (iDA) neuron organoids directly from human fibroblasts is presented. It is confirmed that these 3D iDA organoids more closely resemble the aged human brain and accurately replicate PD pathologies. Furthermore, this model is extended by incorporating astrocytes to create 3D iDA neuron-astrocyte assembloids, recognizing the critical role of glial cells in neurodegenerative processes. It is identified that PD assembloids incorporating control astrocytes with A53T mutant iDAs demonstrated the neuroprotective effects of healthy astrocytes. In contrast, A53T mutant astrocytes progressively contributed to neuronal degeneration and synucleinopathy in 3D-iDA assembloids. These findings suggest that directly converted 3D-iDA organoids and assembloids provide a robust and physiologically relevant model for studying PD pathogenesis and evaluating therapeutic interventions.
帕金森病(PD)的特征是多巴胺能神经元的渐进性丧失和α-突触核蛋白聚集体的积累,但目前的模型无法充分模拟复杂的人类大脑环境。脑类器官模型的最新进展为研究PD提供了一个更具生理相关性的平台,然而,诱导多能干细胞(iPSC)衍生的脑类器官需要很长的成熟时间,可能无法准确代表老年大脑的表观遗传学和细胞背景,限制了它们对PD等晚发性疾病建模的适用性。在本研究中,提出了一种直接从人成纤维细胞生成3D诱导多巴胺能(iDA)神经元类器官的新方法。证实这些3D iDA类器官与老年人类大脑更相似,并能准确复制PD病理。此外,通过纳入星形胶质细胞来创建3D iDA神经元-星形胶质细胞组装体来扩展该模型,认识到神经胶质细胞在神经退行性过程中的关键作用。研究发现,包含对照星形胶质细胞与A53T突变iDA的PD组装体显示出健康星形胶质细胞的神经保护作用。相比之下,A53T突变星形胶质细胞在3D-iDA组装体中逐渐导致神经元变性和突触核蛋白病。这些发现表明,直接转化的3D-iDA类器官和组装体为研究PD发病机制和评估治疗干预提供了一个强大且具有生理相关性的模型。