Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, United States.
Neurosci Lett. 2010 Dec 10;486(2):84-91. doi: 10.1016/j.neulet.2010.08.077. Epub 2010 Sep 17.
Voltage-gated sodium channels are cell membrane glycoproteins responsible for action potential generation and propagation in excitable cells. These large polypeptides which are comprised of 24 transmembrane segments organized into four domains require cellular factors to regulate channel maturation and sorting to different cellular compartments, anchoring the channels at plasma membrane, and modulating gating properties of these channels as effector molecules in the signal transduction pathway. Mutations of sodium channels or their cytosolic partners produce similar pathologies, providing a compelling evidence for the biological significance of channel complexes that form during channel biogenesis and following sorting to different cellular compartments and anchoring at plasma membrane. Genetic, biochemical and bioinformatic approaches have been utilized to identify sodium channel partners. Here we review the important functional role of pan-sodium channel and isoform-specific partners in regulating sodium current density and gating properties.
电压门控钠离子通道是细胞膜糖蛋白，负责可兴奋细胞动作电位的产生和传播。这些由 24 个跨膜片段组成的大型多肽分为四个结构域，需要细胞因子来调节通道的成熟和分拣到不同的细胞区室，将通道锚定在质膜上，并作为信号转导途径中的效应分子调节这些通道的门控特性。钠离子通道或其胞质伴侣的突变产生相似的病理，为通道复合物在通道发生和分拣到不同的细胞区室以及锚定在质膜后的生物发生过程中形成的生物学意义提供了有力证据。遗传、生化和生物信息学方法已被用于鉴定钠离子通道的伴侣。在这里，我们综述了泛钠离子通道和亚型特异性伴侣在调节钠离子电流密度和门控特性方面的重要功能作用。
