The Structure and Function of the Na,K-ATPase Isoforms in Health and Disease.
The Structure and Function of the Na,K-ATPase Isoforms in Health and Disease.
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DOI：
10.3389/fphys.2017.00371
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发表时间：
2017
影响因子：
4
通讯作者：
Poulsen H
中科院分区：
文献类型：
--
作者：
Clausen MV;Hilbers F;Poulsen H
跨质膜的钠和钾梯度被动物细胞用于许多过程，并且需求的范围要求负责的离子泵Na，K-ATP酶可以根据不同的细胞需求进行微调。因此，形成Na，K-ATP酶的三个亚基（α、β和FXYD亚基）中的每一个表达几种同种型。本文综述了Na，K-ATP酶亚基亚型的各种功能和表达模式，并绘制了其序列变异图，以比较结构上的差异。编码α亚单位同种型的Na，K-ATP酶基因突变具有严重的生理后果，导致非常明显的，通常是神经系统疾病。突变的病理生理效应的差异进一步强调了Na，K-ATP酶同工型的动力学参数、调节和蛋白质组相互作用如何针对个体细胞需求进行优化。
The sodium and potassium gradients across the plasma membrane are used by animal cells for numerous processes, and the range of demands requires that the responsible ion pump, the Na,K-ATPase, can be fine-tuned to the different cellular needs. Therefore, several isoforms are expressed of each of the three subunits that make a Na,K-ATPase, the alpha, beta and FXYD subunits. This review summarizes the various roles and expression patterns of the Na,K-ATPase subunit isoforms and maps the sequence variations to compare the differences structurally. Mutations in the Na,K-ATPase genes encoding alpha subunit isoforms have severe physiological consequences, causing very distinct, often neurological diseases. The differences in the pathophysiological effects of mutations further underline how the kinetic parameters, regulation and proteomic interactions of the Na,K-ATPase isoforms are optimized for the individual cellular needs.