Moors Tim E, Milovanovic Dragomir
Ann Romney Center for Neurologic Diseases, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
Laboratory of Molecular Neuroscience, German Center for Neurodegenerative Diseases (DZNE), Berlin, Germany.
J Parkinsons Dis. 2024;14(1):17-33. doi: 10.3233/JPD-230183.
Lewy bodies (LBs) are pathological hallmarks of Parkinson's disease and dementia with Lewy bodies, characterized by the accumulation of α-synuclein (αSyn) protein in the brain. While LBs were first described a century ago, their formation and morphogenesis mechanisms remain incompletely understood. Here, we present a historical overview of LB definitions and highlight the importance of semantic clarity and precise definitions when describing brain inclusions. Recent breakthroughs in imaging revealed shared features within LB subsets and the enrichment of membrane-bound organelles in these structures, challenging the conventional LB formation model. We discuss the involvement of emerging concepts of liquid-liquid phase separation, where biomolecules demix from a solution to form dense condensates, as a potential LB formation mechanism. Finally, we emphasize the need for the operational definitions of LBs based on morphological characteristics and detection protocols, particularly in studies investigating LB formation mechanisms. A better understanding of LB organization and ultrastructure can contribute to the development of targeted therapeutic strategies for synucleinopathies.
路易小体(LBs)是帕金森病和路易体痴呆的病理标志,其特征是大脑中α-突触核蛋白(αSyn)的积累。虽然路易小体在一个世纪前就首次被描述,但它们的形成和形态发生机制仍未完全了解。在这里,我们对路易小体的定义进行了历史概述,并强调了在描述脑内包涵体时语义清晰和精确定义的重要性。成像方面的最新突破揭示了路易小体亚群中的共同特征以及这些结构中膜结合细胞器的富集,这对传统的路易小体形成模型提出了挑战。我们讨论了新兴的液-液相分离概念的参与情况,即生物分子从溶液中分离形成致密凝聚物,这是一种潜在的路易小体形成机制。最后,我们强调需要基于形态特征和检测方案对路易小体进行操作定义,特别是在研究路易小体形成机制的研究中。更好地理解路易小体的组织和超微结构有助于开发针对突触核蛋白病的靶向治疗策略。