皮质Na+通道 (ENaC) 的激活需要CFTR Cl-通道功能
M M Reddy1, M J Light, P M Quinton
1Department of Pediatrics, University of California, San Diego, School of Medicine, La Jolla 92093-0831, USA.
Nature
|December 2, 1999
概括
囊性纤维化会损害盐的吸收,因为减少了通道 (CFTR) 和通道 (ENaC) 的活性. 与以前的观点相反,ENaC功能实际上取决于CFTR活动,揭示了离子运输中的双重缺陷.
科学领域:
- 细胞生理学 细胞生理学
- 离子通道功能的功能
- 遗传疾病 遗传疾病
背景情况:
- 细胞通过协调的离子通道活动来调节电解质运输.
- 囊性纤维化 (CF) 涉及CFTR化通道的损失,影响上皮细胞功能.
- 目前的理解表明,CF气道中的ENaC和CFTR活动是反向的.
研究的目的:
- 调查CFTR和ENAC活动在正常和CF汗道中的关系.
- 为了澄清囊性纤维化中改变吸收的机制.
主要方法:
- 在新分离的正常汗道中研究了离子通道活性.
- 研究了CFTR功能对ENaC活动的影响.
- 在CFTR缺陷的背景下评估Na+行为性.
主要成果:
- 在正常的汗道中,ENaC活动是积极依赖CFTR活动的.
- 发现,囊性纤维化中CFTR缺乏与不可激活的Na+导电性二次相关.
- 证明CF中盐的吸收减少是由于Cl-和Na+导电性受损的结果.
结论:
- 在CFTR和ENaC之间建立的反向关系并不普遍适用.
- 囊性纤维化涉及Na+导电性的二次缺陷,有助于减少盐的吸收.
- 修订了对囊性纤维化病中离子运输失调的理解.
更多相关视频
09:59Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
Published on: March 9, 2015
06:59A Fluorescence-Based Assay of Membrane Potential for High-Throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines
Published on: June 22, 2022
相关概念视频
Ion Channels
The movement of ions like sodium, potassium, and calcium into and out of the cell is essential to maintain the electrochemical gradient in living cells. The ion channels—a class of membrane transport proteins—help maintain this ionic gradient for the smooth functioning of physiological activities such as maintaining cell size and volume, conducting nerve impulses, and gas and nutrient exchange.
Ion channels are specialized integral membrane proteins on the plasma membrane that allow specific...
Ion channels are specialized integral membrane proteins on the plasma membrane that allow specific...
Non-gated Ion Channels
Ion channels are specialized proteins on the plasma membrane that allow charged ions to pass down their electrochemical gradient. Their main function is to maintain the membrane potential which is critical for cell viability. These channels are either gated or non-gated and can transport more than a thousand ions within milliseconds for the cellular event to occur.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism.
Ligand-gated Ion Channels
Ligand-gated ion channels are transmembrane proteins with a channel for ions to pass through and a binding site for a ligand. The channel opens only when a ligand attaches to the binding site.
Three Subfamilies of Ligand-gated Ion Channels
Ligand-gated ion channels fall into three subfamilies. The 'Cys-loop' includes the nicotinic acetylcholine receptors, γ-aminobutyric acid (GABA), glycine, and 5-hydroxytryptamine receptors. The second one is the 'Pore-loop' channels that include the...
Three Subfamilies of Ligand-gated Ion Channels
Ligand-gated ion channels fall into three subfamilies. The 'Cys-loop' includes the nicotinic acetylcholine receptors, γ-aminobutyric acid (GABA), glycine, and 5-hydroxytryptamine receptors. The second one is the 'Pore-loop' channels that include the...
Non-gated Ion Channels
Ion channels are specialized proteins on the plasma membrane that allow charged ions to pass down their electrochemical gradient. Their main function is to maintain the membrane potential which is critical for cell viability. These channels are either gated or non-gated and can transport more than a thousand ions within milliseconds for the cellular event to occur.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism.
Compared to the gated ion channels, the non-gated channels, also known as leakage or passive channels, have no gating mechanism.
Ligand-gated Ion Channels
Ligand-gated ion channels are transmembrane proteins with a channel for ions to pass through and a binding site for a ligand. The channel opens only when a ligand attaches to the binding site.
Three Subfamilies of Ligand-gated Ion Channels
Ligand-gated ion channels fall into three subfamilies. The 'Cys-loop' includes the nicotinic acetylcholine receptors, γ-aminobutyric acid (GABA), glycine, and 5-hydroxytryptamine receptors. The second one is the 'Pore-loop' channels that include the...
Three Subfamilies of Ligand-gated Ion Channels
Ligand-gated ion channels fall into three subfamilies. The 'Cys-loop' includes the nicotinic acetylcholine receptors, γ-aminobutyric acid (GABA), glycine, and 5-hydroxytryptamine receptors. The second one is the 'Pore-loop' channels that include the...
Active Transport
Active transport is a critical biological process that allows cells to move solutes against an electrochemical gradient. This process requires direct energy input and is characterized by its selectivity, saturability, and susceptibility to competitive inhibition.
Primary active transporters, like Na+, K+ and -ATPase, directly utilize ATP to move ions across the membrane. These transporters play significant roles in various physiological processes. For instance, Na+, K+ and -ATPase maintain...
Primary active transporters, like Na+, K+ and -ATPase, directly utilize ATP to move ions across the membrane. These transporters play significant roles in various physiological processes. For instance, Na+, K+ and -ATPase maintain...
