Filamin A in focus: unravelling the multifaceted roles of filamin A in neurodevelopment and neurological disorders.

IF 10.6 1区 医学 Q1 CLINICAL NEUROLOGY
Brain Pub Date : 2025-05-14 DOI:10.1093/brain/awaf180
Longbo Zhang
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引用次数: 0

Abstract

Neurodevelopment is an intricate process encompassing the proliferation, differentiation, migration, and maturation of neural cells. Disruptions in these tightly regulated events can lead to a variety of neurodevelopmental disorders. Filamin A (FLNA), a key actin-binding protein, plays a pivotal role in regulating neuronal migration, morphological development, and synaptic connectivity by modulating actin cytoskeletal dynamics and interacting with various signaling pathways. FLNA mutations are implicated in several neurodevelopmental disorders, such as periventricular nodular heterotopia (PVNH), leading to neurological symptoms such as epilepsy, intellectual disability, and cognitive impairments. In this review, we delve into FLNA's multifaceted role in neurodevelopment, with a particular focus on its contributions to neuronal migration, dendritic and axonal growth, and mechanotransduction. Additionally, we examine how FLNA dysregulation leads to neurodevelopmental abnormalities, providing insights into its potential as a therapeutic target. By elucidating the molecular mechanisms through which FLNA governs neurodevelopment, we aim to advance our understanding of its crucial role in both brain formation and disease pathogenesis.
丝蛋白A聚焦:揭示丝蛋白A在神经发育和神经系统疾病中的多方面作用。
神经发育是一个复杂的过程,包括神经细胞的增殖、分化、迁移和成熟。这些受到严格调控的事件的中断可导致各种神经发育障碍。丝状蛋白A (Filamin A, FLNA)是一种关键的肌动蛋白结合蛋白,通过调节肌动蛋白细胞骨架动力学并与多种信号通路相互作用,在调节神经元迁移、形态发育和突触连接中起关键作用。FLNA突变与几种神经发育障碍有关,如脑室周围结节性异位(PVNH),导致神经系统症状,如癫痫、智力残疾和认知障碍。在这篇综述中,我们深入研究了FLNA在神经发育中的多方面作用,特别关注其对神经元迁移、树突和轴突生长以及机械转导的贡献。此外,我们研究了FLNA失调如何导致神经发育异常,为其作为治疗靶点的潜力提供了见解。通过阐明FLNA调控神经发育的分子机制,我们的目标是推进我们对其在脑形成和疾病发病机制中的关键作用的理解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Brain
Brain 医学-临床神经学
CiteScore
20.30
自引率
4.10%
发文量
458
审稿时长
3-6 weeks
期刊介绍: Brain, a journal focused on clinical neurology and translational neuroscience, has been publishing landmark papers since 1878. The journal aims to expand its scope by including studies that shed light on disease mechanisms and conducting innovative clinical trials for brain disorders. With a wide range of topics covered, the Editorial Board represents the international readership and diverse coverage of the journal. Accepted articles are promptly posted online, typically within a few weeks of acceptance. As of 2022, Brain holds an impressive impact factor of 14.5, according to the Journal Citation Reports.
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