新星:帕金森病α-突触核蛋白淀粉样蛋白聚集的分子机制和化学干预。

IF 4.5 2区 生物学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Shengnan Zhang, Kaien Liu, Dan Li, Cong Liu
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引用次数: 0

摘要

帕金森病(PD)是一种常见的神经退行性疾病,其特征是进行性神经元丧失和α-突触核蛋白(α-syn)病理聚集成淀粉样原纤维,淀粉样原纤维在细胞间繁殖并驱动疾病进展。在过去的十年中,我们的实验室实施了一项综合策略-结合高分辨率结构生物学,分子生物物理学,生化和细胞分析,化学生物学方法和体内疾病模型-来阐明α-syn病理的分子基础。我们首先确定了全长α-syn原纤维的原子分辨率结构,揭示了家族突变和翻译后修饰形成的多种多态性,并将构象异质性与表型和病理多样性联系起来。我们进一步阐明了淀粉样纤维与化学配体相互作用的结构基础,为成像探针和治疗调节剂的合理开发提供了基础。同时,我们发现α-syn原纤维的保守酸性c端区域作为中心界面,驱动病原与多个受体结合,以促进神经传播和炎症诱导,同时还结合自噬接头LC3B,破坏p62介导的选择性自噬。在细胞模型中,用小分子抑制剂靶向该界面可减轻α-syn诱导的毒性。总之,这些发现为了解PD和相关突触核蛋白病的α-syn病理、推进精确诊断和靶向干预提供了一个完整的分子路线图。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Rising Stars: Molecular Mechanisms and Chemical Interventions of α-Synuclein Amyloid Aggregation in Parkinson's Disease.

Parkinson's disease (PD) is a prevalent neurodegenerative disorder characterized by progressive neuronal loss and pathological aggregation of α-synuclein (α-syn) into amyloid fibrils, which propagate between cells and drive disease progression. Over the past decade, our laboratory has implemented an integrated strategy-combining high-resolution structural biology, molecular biophysics, biochemical and cellular analyses, chemical biology approaches, and in vivo disease models-to elucidate the molecular basis of α-syn pathology. We first determined atomic-resolution structures of full-length α-syn fibrils, revealing diverse polymorphs shaped by familial mutations and post-translational modifications, and linking conformational heterogeneity to phenotypic and pathological diversity. We further elucidated the structural basis underlying the interaction between amyloid fibril and chemical ligands, enabling the rational development of imaging probes and therapeutic modulators. In parallel, we found that the conserved acidic C-terminal region of α-syn fibrils acts as a central interface for driving pathogenic engagement with multiple receptors for neural propagation and inflammation induction, while also binding the autophagy adaptor LC3B to disrupt p62-mediated selective autophagy. Targeting this interface with small molecule inhibitors alleviates α-syn-induced toxicity in cellular models. Together, these findings provide an integrated molecular roadmap for understanding α-syn pathology and advancing precision diagnostics and targeted interventions in PD and related synucleinopathies.

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来源期刊
Journal of Molecular Biology
Journal of Molecular Biology 生物-生化与分子生物学
CiteScore
11.30
自引率
1.80%
发文量
412
审稿时长
28 days
期刊介绍: Journal of Molecular Biology (JMB) provides high quality, comprehensive and broad coverage in all areas of molecular biology. The journal publishes original scientific research papers that provide mechanistic and functional insights and report a significant advance to the field. The journal encourages the submission of multidisciplinary studies that use complementary experimental and computational approaches to address challenging biological questions. Research areas include but are not limited to: Biomolecular interactions, signaling networks, systems biology; Cell cycle, cell growth, cell differentiation; Cell death, autophagy; Cell signaling and regulation; Chemical biology; Computational biology, in combination with experimental studies; DNA replication, repair, and recombination; Development, regenerative biology, mechanistic and functional studies of stem cells; Epigenetics, chromatin structure and function; Gene expression; Membrane processes, cell surface proteins and cell-cell interactions; Methodological advances, both experimental and theoretical, including databases; Microbiology, virology, and interactions with the host or environment; Microbiota mechanistic and functional studies; Nuclear organization; Post-translational modifications, proteomics; Processing and function of biologically important macromolecules and complexes; Molecular basis of disease; RNA processing, structure and functions of non-coding RNAs, transcription; Sorting, spatiotemporal organization, trafficking; Structural biology; Synthetic biology; Translation, protein folding, chaperones, protein degradation and quality control.
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