Publications:

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Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.

The Journal Of Physiology

Yeh, Jiunn-Tyng JT; Yu, Ying-Chun YC; Hwang, Tzyh-Chang TC

Publication Date: 2019-01

Variant appearance in text: CFTR: Y1219G

PubMed Link: 30408177

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Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.

Molecular Pharmacology

Lin, Wen-Ying WY; Sohma, Yoshiro Y; Hwang, Tzyh-Chang TC

Publication Date: 2016-09

Variant appearance in text: CFTR: Y1219G

PubMed Link: 27413118

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Long-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channels.

Faseb Journal : Official Publication Of The Federation Of American Societies For Experimental Biology

Wei, Shipeng S; Roessler, Bryan C BC; Icyuz, Mert M; Chauvet, Sylvain S; Tao, Binli B; Hartman, John L JL; Kirk, Kevin L KL

Publication Date: 2016-03

Variant appearance in text: CFTR: Y1219G

PubMed Link: 26606940

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The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.

Pflugers Archiv : European Journal Of Physiology

Broadbent, Steven D SD; Ramjeesingh, Mohabir M; Bear, Christine E CE; Argent, Barry E BE; Linsdell, Paul P; Gray, Michael A MA

Publication Date: 2015-08

Variant appearance in text: CFTR: Y1219G

PubMed Link: 25277268

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• 424_2014_Article_1618.pdf

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A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.

The Journal Of General Physiology

Lin, Wen-Ying WY; Jih, Kang-Yang KY; Hwang, Tzyh-Chang TC

Publication Date: 2014-10

Variant appearance in text: CFTR: Y1219G

PubMed Link: 25225552

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• JGP_201411247.pdf

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Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.

The Journal Of Biological Chemistry

Wei, Shipeng S; Roessler, Bryan C BC; Chauvet, Sylvain S; Guo, Jingyu J; Hartman, John L JL; Kirk, Kevin L KL

Publication Date: 2014-07-18

Variant appearance in text: CFTR: Y1219G

PubMed Link: 24876383

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Optimization of the degenerated interfacial ATP binding site improves the function of disease-related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels.

The Journal Of Biological Chemistry

Tsai, Ming-Feng MF; Jih, Kang-Yang KY; Shimizu, Hiroyasu H; Li, Min M; Hwang, Tzyh-Chang TC

Publication Date: 2010-11-26

Variant appearance in text: CFTR: Y1219G

PubMed Link: 20861014

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A stable ATP binding to the nucleotide binding domain is important for reliable gating cycle in an ABC transporter CFTR.

The Journal Of Physiological Sciences : Jps

Shimizu, Hiroyasu H; Yu, Ying-Chun YC; Kono, Koichi K; Kubota, Takahiro T; Yasui, Masato M; Li, Min M; Hwang, Tzyh-Chang TC; Sohma, Yoshiro Y

Publication Date: 2010-09

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PubMed Link: 20628841

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Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel.

The Journal Of General Physiology

Tsai, Ming-Feng MF; Li, Min M; Hwang, Tzyh-Chang TC

Publication Date: 2010-05

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PubMed Link: 20421370

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Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs.

The Journal Of Biological Chemistry

Miki, Haruna H; Zhou, Zhen Z; Li, Min M; Hwang, Tzyh-Chang TC; Bompadre, Silvia G SG

Publication Date: 2010-06-25

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PubMed Link: 20406820

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State-dependent modulation of CFTR gating by pyrophosphate.

The Journal Of General Physiology

Tsai, Ming-Feng MF; Shimizu, Hiroyasu H; Sohma, Yoshiro Y; Li, Min M; Hwang, Tzyh-Chang TC

Publication Date: 2009-04

Variant appearance in text: CFTR: Y1219G

PubMed Link: 19332621

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Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation.

The Journal Of Physiology

Hwang, Tzyh-Chang TC; Sheppard, David N DN

Publication Date: 2009-05-15

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PubMed Link: 19332488

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Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.

Philosophical Transactions Of The Royal Society Of London. Series B, Biological Sciences

Muallem, Daniella D; Vergani, Paola P

Publication Date: 2009-01-27

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PubMed Link: 18957373

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G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects.

The Journal Of General Physiology

Bompadre, Silvia G SG; Sohma, Yoshiro Y; Li, Min M; Hwang, Tzyh-Chang TC

Publication Date: 2007-04

Variant appearance in text: CFTR: Y1219G

PubMed Link: 17353351

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• jgp1290285.pdf

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The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics.

The Journal Of General Physiology

Zhou, Zhen Z; Wang, Xiaohui X; Liu, Hao-Yang HY; Zou, Xiaoqin X; Li, Min M; Hwang, Tzyh-Chang TC

Publication Date: 2006-10

Variant appearance in text: CFTR: Y1219G

PubMed Link: 16966475

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