Animal models of focal cortical dysplasia and tuberous sclerosis complex: recent progress toward clinical applications.
Animal models of focal cortical dysplasia and tuberous sclerosis complex: recent progress toward clinical applications.
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DOI：
10.1111/j.1528-1167.2009.02295.x
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发表时间：
2009-10
期刊：
影响因子：
5.6
通讯作者：
Wong M
中科院分区：
文献类型：
--
作者：
Wong M
局灶性皮质发育不良（FCD）和相关的皮质发育畸形（MCD）代表了越来越多的公认的医学难治性癫痫的原因。然而，癫痫发生的潜在机制知之甚少，由于各种皮质畸形导致的癫痫治疗往往有限或无效。动物模型为研究导致癫痫发生的细胞和分子机制以及为MCD相关癫痫开发新的合理疗法提供了许多优势。本文将重点介绍动物模型如何用于解决FCD和相关皮质畸形所致癫痫的几个临床相关问题，包括病理和临床特征、病因、致痫区定位、神经元和星形胶质细胞对癫痫发生的贡献以及抗癫痫治疗的发展。
Focal cortical dysplasia (FCD) and related malformations of cortical development (MCD) represent an increasingly recognized cause of medically-intractable epilepsy. However, the underlying mechanisms of epileptogenesis are poorly understood, and treatments for epilepsy due to various cortical malformations are often limited or ineffective. Animal models offer a number of advantages for investigating cellular and molecular mechanisms causing epileptogenesis and developing novel, rational therapies for MCD-related epilepsy. This review will highlight specific examples of how animal models have been useful in addressing several clinically-relevant issues about epilepsy due to FCDs and related cortical malformations, including the pathological and clinical features, etiological factors, localization of the epileptogenic zone, neuronal and astrocytic contributions to epileptogenesis, and the development of anti-epileptogenic therapies.
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影响因子：
14.5
作者：
Alonso-Nanclares, L;Garbelli, R;DeFelipe, J
通讯作者：
DeFelipe, J
影响因子：
4.3
作者：
Ljungberg, M. Cecilia;Sunnen, C. Nicole;D'Arcangelo, Gabriella
通讯作者：
D'Arcangelo, Gabriella
影响因子：
2.2
作者：
Baraban, SC;Schwartzkroin, PA
通讯作者：
Schwartzkroin, PA
影响因子：
2.2
作者：
Baraban, SC;Schwartzkroin, PA
通讯作者：
Schwartzkroin, PA
影响因子：
5.6
作者：
Harrington, Emily P.;Moddel, Gabriel;Baraban, Scott C.
通讯作者：
Baraban, Scott C.