囊性纤维化跨膜电导调节因子（ABCC7）结构。
Cystic fibrosis transmembrane conductance regulator (ABCC7) structure.
机构信息
Department of Biological Sciences, Columbia University, New York, NY 10027, USA.
出版信息
Cold Spring Harb Perspect Med. 2013 Feb 1;3(2):a009514. doi: 10.1101/cshperspect.a009514.
Abstract
Structural studies of the cystic fibrosis transmembrane conductance regulator (CFTR) are reviewed. Like many membrane proteins, full-length CFTR has proven to be difficult to express and purify, hence much of the structural data available is for the more tractable, independently expressed soluble domains. Therefore, this chapter covers structural data for individual CFTR domains in addition to the sparser data available for the full-length protein. To set the context for these studies, we will start by reviewing structural information on model proteins from the ATP-binding cassette (ABC) transporter superfamily, to which CFTR belongs.
摘要
对囊性纤维化跨膜电导调节因子（CFTR）的结构研究进行了综述。与许多膜蛋白一样，全长 CFTR 的表达和纯化证明极具挑战性，因此现有的大量结构数据都来自于更易于处理的独立表达的可溶性结构域。因此，本章除了涵盖全长蛋白的可用数据更为稀疏的内容外，还涵盖了各个 CFTR 结构域的结构数据。为了给这些研究提供背景信息，我们将首先回顾 ABC 转运蛋白超家族（CFTR 所属的家族）中模型蛋白的结构信息。
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2
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J Chem Inf Model. 2012 Jul 23;52(7):1842-53. doi: 10.1021/ci2005884. Epub 2012 Jul 12.
3
Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.变构调节平衡热力学稳定性并恢复 ΔF508 CFTR 的功能。
J Mol Biol. 2012 May 25;419(1-2):41-60. doi: 10.1016/j.jmb.2012.03.001. Epub 2012 Mar 8.
4
Requirements for efficient correction of ΔF508 CFTR revealed by analyses of evolved sequences.通过对进化序列的分析揭示了 ΔF508 CFTR 有效校正的要求。
Cell. 2012 Jan 20;148(1-2):164-74. doi: 10.1016/j.cell.2011.11.023.
5
Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.纠正 NBD1 的能量和结构域界面是恢复 ΔF508 CFTR 折叠和功能所必需的。
Cell. 2012 Jan 20;148(1-2):150-63. doi: 10.1016/j.cell.2011.11.024.
6
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Biochemistry. 2011 Nov 29;50(47):10311-7. doi: 10.1021/bi201176q. Epub 2011 Nov 4.
7
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Essays Biochem. 2011 Sep 7;50(1):43-61. doi: 10.1042/bse0500043.
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J Biol Chem. 2011 Dec 9;286(49):42647-42654. doi: 10.1074/jbc.M111.292268. Epub 2011 Sep 19.
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