期刊
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
卷 42, 期 7, 页码 593-606出版社
WILEY-BLACKWELL
DOI: 10.1111/nan.12331
关键词
astrocyte; cortical dysplasia; epilepsy; glial dysfunction; gliosis
资金
- Polish National Science Centre (NCN) [2012/05/N/NZ2/00641, 2011/01/B/NZ4/00586]
- Jagiellonian University Faculty of Biology and Earth Sciences grant [DS/MND/WBiNoZ/IZ/23/2013]
Malformations of cortical development (MCDs), such as cortical dysplasia and tuberous sclerosis complex, are common causes of intractable epilepsy, especially in paediatric patients. Recently, mounting evidence points to a common pathology of these disorders. Hyperactivation of mammalian target of rapamycin (mTOR) has been proposed as a central mechanism in most, if not all, MCDs. The transition from mTOR hyperactivation and cellular abnormalities to large-scale functional changes and seizure is, however, not fully understood. In this article we set out to review currently available information regarding MCD pathology, focusing on glial cells - especially astrocytes - and their interactions with the brain vascular system. A large body of evidence points to these elements as potential targets in MCD. Here, we attempt to provide a review of this evidence and propose some hypotheses regarding the possible chain of events linking primary glial dysfunction and epilepsy. We focus on extracellular matrix remodelling, blood-brain barrier leakage and failure of astrocyte-dependent removal of extracellular debris. We posit that the failure of these systems results in a chronically pro-inflammatory environment, maintaining local astrocytes in a state of gliosis, with increased susceptibility to seizures as a consequence.
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