Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
about
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTRSynergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.Mutation-specific downregulation of CFTR2 variants by gating potentiators.
P2860
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P2860
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
description
2014 nî lūn-bûn
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2014年の論文
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2014年論文
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2014年論文
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2014年論文
@zh-hk
2014年論文
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2014年論文
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2014年论文
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2014年论文
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2014年论文
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name
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@en
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@nl
type
label
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@en
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@nl
prefLabel
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@en
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@nl
P2093
P2860
P1476
Revertant mutants modify, but ...... ic fibrosis mutant G551D-CFTR.
@en
P2093
Carlos M Farinha
David N Sheppard
Luísa S Pissarra
Margarida D Amaral
Patrick H Thibodeau
Zhiwei Cai
P2860
P304
P356
10.1113/JPHYSIOL.2014.271817
P407
P577
2014-03-03T00:00:00Z