癫痫
加巴能
γ-氨基丁酸受体
协同运输机
神经科学
γ-氨基丁酸
癫痫发生
受体
医学
生物
内科学
抑制性突触后电位
化学
有机化学
钠
作者
Yvonne E. Moore,Matthew R. Kelley,Nicholas J. Brandon,Tarek Z. Deeb,Stephen J. Moss
标识
DOI:10.1016/j.tins.2017.06.008
摘要
Phosphoregulation supersedes the classic ways of thinking about transporters (total and surface numbers). Type 2 K+–Cl− cotransporter (KCC2) loss-of-function mutations cause infantile epilepsy. Idiopathic and acquired epilepsy both involve downregulation of KCC2. KCC2 is dysfunctional in neurodevelopmental disorders that are associated with seizures. Deficits in GABAergic inhibition result in the abnormal neuronal activation and synchronization that underlies seizures. However, the molecular mechanisms responsible for transforming a normal brain into an epileptic one remain largely unknown. Hyperpolarizing inhibition mediated by type A GABA (GABAA) receptors is dependent on chloride extrusion by the neuron-specific type 2 K+–Cl− cotransporter (KCC2). Loss-of-function mutations in KCC2 are a known cause of infantile epilepsy in humans and KCC2 dysfunction is present in patients with both idiopathic and acquired epilepsy. Here we discuss the growing evidence that KCC2 dysfunction has a central role in the development and severity of the epilepsies. Deficits in GABAergic inhibition result in the abnormal neuronal activation and synchronization that underlies seizures. However, the molecular mechanisms responsible for transforming a normal brain into an epileptic one remain largely unknown. Hyperpolarizing inhibition mediated by type A GABA (GABAA) receptors is dependent on chloride extrusion by the neuron-specific type 2 K+–Cl− cotransporter (KCC2). Loss-of-function mutations in KCC2 are a known cause of infantile epilepsy in humans and KCC2 dysfunction is present in patients with both idiopathic and acquired epilepsy. Here we discuss the growing evidence that KCC2 dysfunction has a central role in the development and severity of the epilepsies.
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