Weiergräber M, Hescheler J, Schneider T
Institut für Neurophysiologie, Medizinische Einrichtungen, Universität zu Köln, Robert-Koch-Strasse 39, 50931, Köln, Deutschland.
Nervenarzt. 2008 Apr;79(4):426-36. doi: 10.1007/s00115-007-2398-6.
Voltage-gated calcium channels are key components in a variety of physiological processes. Within the last decade an increasing number of voltage-gated Ca(2+) channelopathies in both humans and animal models has been described, most of which are related to the neurologic and muscular system. In humans, mutations were found in L-type Ca(v)1.2 and Ca(v)1.4 Ca(2+) channels as well as the non-L-type Ca(v)2.1 and T-type Ca(v)3.2 channels, resulting in altered electrophysiologic properties. Based on their widespread distribution within the CNS, voltage-gated calcium channels are of particular importance in the etiology and pathogenesis of various forms of epilepsy and neuropsychiatric disorders. In this review we characterise the different human Ca(2+) channelopathies known so far, further illuminating basic pathophysiologic mechanisms and clinical aspects.
电压门控钙通道是多种生理过程中的关键组成部分。在过去十年中,已描述了人类和动物模型中越来越多的电压门控Ca(2+)通道病,其中大多数与神经和肌肉系统有关。在人类中,发现L型Ca(v)1.2和Ca(v)1.4 Ca(2+)通道以及非L型Ca(v)2.1和T型Ca(v)3.2通道发生突变,导致电生理特性改变。基于它们在中枢神经系统中的广泛分布,电压门控钙通道在各种形式的癫痫和神经精神疾病的病因和发病机制中尤为重要。在本综述中,我们对目前已知的不同人类Ca(2+)通道病进行了特征描述,进一步阐明了基本的病理生理机制和临床方面。