摘要
K+ 通道在所有可兴奋性和非兴奋性细胞的重要信号传导过程中扮演着重要角色 ,其家族成员在调节神经递质释放、心率、胰岛素分泌、神经细胞分泌、上皮细胞电传导、骨骼肌收缩以及细胞容积的过程中发挥了重要作用 .在编码K+通道的基因发生突变的情况下 ,可引起 K+通道病 .Andersen's(AS)综合征由于在表达 Kir2 .1的 K CN J2基因上发生了突变 ,而 Kir2 .1是 K+ 通道的 1个α亚基 ,它在很多类型细胞中可以决定并稳定静息膜电位 .这种罕见的家族性疾病以 3个主要的临床症状为特征 :骨结构发育不良、周期性麻痹和心率不齐 .我们将从 3个方面阐述上述内容 :K+通道的结构 ,特别是 Kir家族 ;K+ 通道病 ;Kir2 .1与
Potassium channels play important roles in vital cellular signaling processes in both excitable and nonexcitable cells. Members of this channel family play critical roles in cellular signaling processes regulating neurotransmitter release, heart rate, insulin secretion, neuronal excitability, epithelial electrolyte transport, smooth muscle contraction, and cell volume regulation. The mutations in genes encoding K+ channels can cause potassium channelopathies. Andersen's syndrome is caused by mutations in the KCNJ 2 gene, which encodes Kir2.1, an α subunit of a potassium channel, and is involved in determining and stabilizing the resting membrane potential in many cell types. This rare familial disease characterized by three key clinical features developmental defects in bony structure, periodic paralysis and cardiac arrhythmias. This review will deal with the structure of potassium channels, especially the Kir family, potassium channelopathesis, Kir2.1 and Andersen's syndrome.
出处
《第四军医大学学报》
北大核心
2002年第22期2108-2111,共4页
Journal of the Fourth Military Medical University
