Nägerl U V, Mody I, Jeub M, Lie A A, Elger C E, Beck H
Department of Neurology, University of California at Los Angeles School of Medicine, Los Angeles, California 90095, USA.
J Neurosci. 2000 Mar 1;20(5):1831-6. doi: 10.1523/JNEUROSCI.20-05-01831.2000.
In mesial temporal lobe epilepsy (mTLE), the predominant form of epilepsy in adults, and in animal models of the disease, there is a conspicuous loss of the intracellular Ca(2+)-binding protein calbindin-D(28k) (CB) from granule cells (GCs) of the dentate gyrus. The role of this protein in nerve cell function is controversial, but here we provide evidence for its role in controlling Ca(2+) influx into human neurons. In patients with Ammon's horn sclerosis (AHS), the loss of CB from GCs markedly increased the Ca(2+)-dependent inactivation of voltage-dependent Ca(2+) currents (I(Ca)), thereby diminishing Ca(2+) influx during repetitive neuronal firing. Introducing purified CB into GCs restored Ca(2+) current inactivation to levels observed in cells with normal CB content harvested from mTLE patients without AHS. Our data are consistent with the possibility of neuroprotection secondary to the CB loss. By limiting Ca(2+) influx through an enhanced Ca(2+)-dependent inactivation of voltage-dependent Ca(2+) channels during prolonged neuronal discharges, the loss of CB may contribute to the resistance of surviving human granule cells in AHS.
在成人癫痫的主要形式——内侧颞叶癫痫(mTLE)以及该疾病的动物模型中,齿状回颗粒细胞(GCs)内的细胞内钙结合蛋白钙结合蛋白-D(28k)(CB)明显缺失。这种蛋白质在神经细胞功能中的作用存在争议,但在此我们提供了其在控制钙离子流入人类神经元中作用的证据。在海马硬化(AHS)患者中,GCs中CB的缺失显著增加了电压依赖性钙电流(I(Ca))的钙依赖性失活,从而减少了重复神经元放电期间的钙离子流入。将纯化的CB引入GCs可使钙电流失活恢复到从无AHS的mTLE患者中获取的CB含量正常的细胞中观察到的水平。我们的数据与CB缺失继发神经保护作用的可能性一致。通过在长时间神经元放电期间通过增强电压依赖性钙通道的钙依赖性失活来限制钙离子流入,CB的缺失可能有助于AHS中存活的人类颗粒细胞的抗性。