丙戊酸体外模型评估神经发育毒性:范围综述。

IF 6.6 1区 医学 Q1 CLINICAL NEUROLOGY
Epilepsia Pub Date : 2025-03-28 DOI:10.1111/epi.18392
Daniel Sandvik, Elena Vianca, Alison Anderson, Muhammad Shahid Javaid, Terence J O'Brien, Ana Antonic-Baker
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引用次数: 0

摘要

丙戊酸(VPA)是一种治疗全身性和局灶性癫痫的一线抗癫痫药物(ASM)。不幸的是,由于其与致畸作用的强烈关联,最终导致胎儿丙戊酸谱系障碍(FVSD),其中可能包括神经认知和神经行为缺陷,因此该药物已被高度管制/限制用于有生育潜力的妇女。这包括那些已被证明对药物反应良好而对其他asm反应不佳的人。同时,VPA的神经毒性和致畸机制已经在体外进行了研究,持续的研究可能有助于我们深入了解,以便为癫痫患者制定优质的循证护理计划和新型药物设计。本综述系统地评估了体外研究发现的VPA对神经细胞的影响以及提出的细胞神经毒性机制。神经毒性研究已经捕获了小鼠和人类原代、永生化和体外干细胞的细胞毒性、畸形、遗传和表观遗传效应。这包括与神经发育障碍相关的许多基因和基因通路的广泛鉴定,这是FVSD的一个标志。尽管已发表的研究已经阐明了VPA的神经毒性和致畸作用,但缺乏标准化的测试方法使得直接比较不同研究的结果具有挑战性。然而,最近使用的基于人类干细胞的模型为VPA暴露引起的细胞、分子、遗传和表观遗传效应提供了更丰富的理解。未来的体外研究可能会通过给人干细胞来源的神经细胞注射临床相关浓度的VPA来提高其临床可翻译性,并促进对VPA神经细胞类型特异性和表观遗传效应的更好理解。体外VPA神经毒性研究对神经发育的影响显示出明确的数据潜力,可能有助于为具有生育能力的癫痫障碍妇女构建卓越的个性化循证治疗计划和新型药物设计。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
In vitro models of valproic acid to assess neurodevelopmental toxicity: A scoping review.

Valproic acid (VPA) is a first-line antiseizure medication (ASM) that is highly efficacious for treating generalized and focal epilepsy disorders. Unfortunately, due to its strong association with teratogenic effects culminating in fetal valproate spectrum disorder (FVSD), which may include neurocognitive and neurobehavioral deficits, the drug has become highly regulated/restricted for women of childbearing potential. This includes those who have been shown to respond well to the drug and respond poorly to alternative ASMs. Concurrently, VPA's neurotoxic, teratogenic mechanisms have been studied in vitro, and continued research may aid in providing depth to our understanding so that superior evidence-based care plans and novel drug designs can be made for patients with epilepsy disorders. This scoping review systematically assesses what in vitro studies have discovered regarding VPA's effects on neural cells and the proposed cellular neurotoxic mechanisms. Neurotoxicity studies have captured the cytotoxic, dysmorphological, genetic, and epigenetic effects in murine and human primary, immortalized, and stem cells in vitro. This includes extensive identification of many genes and gene pathways associated with neurodevelopmental disorders, a hallmark of FVSD. Although published studies have illuminated much about VPA's neurotoxic, teratogenic effects, a lack of standardization in testing methodologies renders making direct comparisons between the results of different studies challenging. Nevertheless, the recent use of human stem cell-based models provides a richer understanding of what cellular, molecular, genetic, and epigenetic effects are caused by VPA exposure. Future in vitro studies may improve their clinical translatability by administering clinically relevant concentrations of VPA to human stem cell-derived neural cells and fostering a better understanding of VPA's neural cell type-specific and epigenetic effects. In vitro VPA neurotoxicity studies on neurodevelopment show a clear potential to provide data that may help construct superior personalized evidence-based treatment plans and novel drug designs for women of childbearing potential with epilepsy disorders.

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来源期刊
Epilepsia
Epilepsia 医学-临床神经学
CiteScore
10.90
自引率
10.70%
发文量
319
审稿时长
2-4 weeks
期刊介绍: Epilepsia is the leading, authoritative source for innovative clinical and basic science research for all aspects of epilepsy and seizures. In addition, Epilepsia publishes critical reviews, opinion pieces, and guidelines that foster understanding and aim to improve the diagnosis and treatment of people with seizures and epilepsy.
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