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

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 signalling 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.

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Publication Details

Journal
Brain
Published
2025-05-14
DOI
https://doi.org/10.1093/brain/awaf180
Citations
9
Primary Topic
Fetal and Pediatric Neurological Disorders
Type
article
Field-Weighted Citation Impact
31.45

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article

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

Longbo Zhang
9 citations
Brain
Fetal and Pediatric Neurological Disorders
31.45
article

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

Longbo Zhang
article en
9 citations

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 signalling 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.

BrainVol. 148(10)
Central South University (CN), Yale University (US), National Clinical Research (US), Xiangya Hospital Central South University (CN)
Central South University
Reduced inequalities
Openalex Percentile: Top 0%
Fetal and Pediatric Neurological Disorders
31.45
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