Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1
出版年份 2010 全文链接
标题
Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1
作者
关键词
-
出版物
PROTEIN SCIENCE
Volume 19, Issue 10, Pages 1917-1931
出版商
Wiley
发表日期
2010-08-05
DOI
10.1002/pro.479
参考文献
相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。- Regulatory Insertion Removal Restores Maturation, Stability and Function of ΔF508 CFTR
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- Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant
- (2010) Shane Atwell et al. PROTEIN ENGINEERING DESIGN & SELECTION
- Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis
- (2010) Chi Wang et al. PROTEIN SCIENCE
- Thermal stability of α-amylase in aqueous cosolvent systems
- (2009) Jay Kant Yadav et al. JOURNAL OF BIOSCIENCES
- Structure and Dynamics of NBD1 from CFTR Characterized Using Crystallography and Hydrogen/Deuterium Exchange Mass Spectrometry
- (2009) H.A. Lewis et al. JOURNAL OF MOLECULAR BIOLOGY
- Cooperative Assembly and Misfolding of CFTR Domains In Vivo
- (2009) Kai Du et al. MOLECULAR BIOLOGY OF THE CELL
- CFTR Function and Prospects for Therapy
- (2008) John R. Riordan Annual Review of Biochemistry
- Correctors promote folding of the CFTR in the endoplasmic reticulum
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- Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
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- Misfolding of the Cystic Fibrosis Transmembrane Conductance Regulator and Disease†
- (2008) Joanne C. Cheung et al. BIOCHEMISTRY
- Thermodynamic and kinetic stability of penicillin acylase from Escherichia coli
- (2008) Valerij Ya. Grinberg et al. BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS
- Atomic model of human cystic fibrosis transmembrane conductance regulator: Membrane-spanning domains and coupling interfaces
- (2008) J.-P. Mornon et al. CELLULAR AND MOLECULAR LIFE SCIENCES
- Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation
- (2008) Luísa S. Pissarra et al. CHEMISTRY & BIOLOGY
- Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
- (2008) A. W. R. Serohijos et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Chemical and Biological Folding Contribute to Temperature-Sensitive ΔF508 CFTR Trafficking
- (2008) Xiaodong Wang et al. TRAFFIC
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