Sen Arjune, Thom Maria, Nikolić Margareta, Sisodiya Sanjay M
Department of Clinical and Experimental Epilepsy, University College London, London, UK.
Dev Neurosci. 2008;30(1-3):96-104. doi: 10.1159/000109855.
Focal cortical dysplasia (FCD) is the most common malformation of cortical development found in epilepsy surgical series. Characterised by cortical mislamination, dysplastic neurons and, in a subgroup of cases, balloon cells, FCD is potently epileptogenic. Despite decades of study, the underlying aetiology of FCD remains uncertain and research has been hampered by the lack of a good animal model in which to simulate the condition. In this article we review some of the potential molecular mechanisms that might underpin human FCD. In particular we examine the potential role of cyclin-dependent kinase 5 and its principal activator p35 in FCD and estimate the contribution that deregulation of cyclin-dependent kinase 5 might make to the pathogenesis of this condition.
局灶性皮质发育不良（FCD）是癫痫手术病例中最常见的皮质发育畸形。FCD的特征是皮质结构紊乱、神经元发育异常，在一部分病例中还存在气球样细胞，具有很强的致痫性。尽管经过了数十年的研究，FCD的潜在病因仍不明确，且由于缺乏能够模拟该病症的良好动物模型，研究受到了阻碍。在本文中，我们综述了一些可能构成人类FCD基础的潜在分子机制。特别地，我们研究了细胞周期蛋白依赖性激酶5及其主要激活剂p35在FCD中的潜在作用，并评估细胞周期蛋白依赖性激酶5失调可能对该病症发病机制产生的影响。
