Publications:

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MetalProGNet: a structure-based deep graph model for metalloprotein-ligand interaction predictions.

Chemical Science

Jiang, Dejun D; Ye, Zhaofeng Z; Hsieh, Chang-Yu CY; Yang, Ziyi Z; Zhang, Xujun X; Kang, Yu Y; Du, Hongyan H; Wu, Zhenxing Z; Wang, Jike J; Zeng, Yundian Y; Zhang, Haotian H; Wang, Xiaorui X; Wang, Mingyang M; Yao, Xiaojun X; Zhang, Shengyu S; Wu, Jian J; Hou, Tingjun T

Publication Date: 2023-02-22

Variant appearance in text: CFTR: Q637R

PubMed Link: 36845922

Variant Present in the following documents:

• SC-014-D2SC06576B-s003.xlsx, sheet 1
• SC-014-D2SC06576B-s002.xlsx, sheet 1

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Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants.

International Journal Of Molecular Sciences

Baatallah, Nesrine N; Elbahnsi, Ahmad A; Chevalier, Benoit B; Castanier, Solène S; Mornon, Jean-Paul JP; Pranke, Iwona I; Edelman, Aleksander A; Sermet-Gaudelus, Isabelle I; Callebaut, Isabelle I; Hinzpeter, Alexandre A

Publication Date: 2022-12-19

Variant appearance in text: CFTR: Q637R

PubMed Link: 36555865

Variant Present in the following documents:

• Main text
• ijms-23-16225.pdf

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Nanomechanics combined with HDX reveals allosteric drug binding sites of CFTR NBD1.

Computational And Structural Biotechnology Journal

Padányi, Rita R; Farkas, Bianka B; Tordai, Hedvig H; Kiss, Bálint B; Grubmüller, Helmut H; Soya, Naoto N; Lukács, Gergely L GL; Kellermayer, Miklós M; Hegedűs, Tamás T

Publication Date: 2022

Variant appearance in text: CFTR: Q637R

PubMed Link: 35685375

Variant Present in the following documents:

• mmc1.pdf

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A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease.

The Embo Journal

Villella, Valeria R VR; Venerando, Andrea A; Cozza, Giorgio G; Esposito, Speranza S; Ferrari, Eleonora E; Monzani, Romina R; Spinella, Mara C MC; Oikonomou, Vasilis V; Renga, Giorgia G; Tosco, Antonella A; Rossin, Federica F; Guido, Stefano S; Silano, Marco M; Garaci, Enrico E; Chao, Yu-Kai YK; Grimm, Christian C; Luciani, Alessandro A; Romani, Luigina L; Piacentini, Mauro M; Raia, Valeria V; Kroemer, Guido G; Maiuri, Luigi L

Publication Date: 2019-01-15

Variant appearance in text: CFTR: Q637R

PubMed Link: 30498130

Variant Present in the following documents:

• Main text
• EMBJ-38-e100101.pdf

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Differential Scanning Fluorimetry and Hydrogen Deuterium Exchange Mass Spectrometry to Monitor the Conformational Dynamics of NBD1 in Cystic Fibrosis.

Methods In Molecular Biology (Clifton, N.J.)

Soya, Naoto N; Roldan, Ariel A; Lukacs, Gergely L GL

Publication Date: 2019

Variant appearance in text: CFTR: Q637R

PubMed Link: 30341603

Variant Present in the following documents:

• Main text

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Structure-guided combination therapy to potently improve the function of mutant CFTRs.

Nature Medicine

Veit, Guido G; Xu, Haijin H; Dreano, Elise E; Avramescu, Radu G RG; Bagdany, Miklos M; Beitel, Lenore K LK; Roldan, Ariel A; Hancock, Mark A MA; Lay, Cecilia C; Li, Wei W; Morin, Katelin K; Gao, Sandra S; Mak, Puiying A PA; Ainscow, Edward E; Orth, Anthony P AP; McNamara, Peter P; Edelman, Aleksander A; Frenkiel, Saul S; Matouk, Elias E; Sermet-Gaudelus, Isabelle I; Barnes, William G WG; Lukacs, Gergely L GL

Publication Date: 2018-11

Variant appearance in text: CFTR: Q637R

PubMed Link: 30297908

Variant Present in the following documents:

• Main text
• NIHMS1503238-supplement-2.pdf
• nihms-1503238.pdf

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Sites associated with Kalydeco binding on human Cystic Fibrosis Transmembrane Conductance Regulator revealed by Hydrogen/Deuterium Exchange.

Scientific Reports

Byrnes, Laura J LJ; Xu, Yingrong Y; Qiu, Xiayang X; Hall, Justin D JD; West, Graham M GM

Publication Date: 2018-03-16

Variant appearance in text: CFTR: Q637R

PubMed Link: 29549268

Variant Present in the following documents:

• 41598_2018_Article_22959.pdf

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Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.

Biochimica Et Biophysica Acta. Biomembranes

Yang, Zhengrong Z; Hildebrandt, Ellen E; Jiang, Fan F; Aleksandrov, Andrei A AA; Khazanov, Netaly N; Zhou, Qingxian Q; An, Jianli J; Mezzell, Andrew T AT; Xavier, Bala M BM; Ding, Haitao H; Riordan, John R JR; Senderowitz, Hanoch H; Kappes, John C JC; Brouillette, Christie G CG; Urbatsch, Ina L IL

Publication Date: 2018-05

Variant appearance in text: CFTR: Q637R

PubMed Link: 29425673

Variant Present in the following documents:

• Main text

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Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.

Nature Communications

Bagdany, Miklos M; Veit, Guido G; Fukuda, Ryosuke R; Avramescu, Radu G RG; Okiyoneda, Tsukasa T; Baaklini, Imad I; Singh, Jay J; Sovak, Guy G; Xu, Haijin H; Apaja, Pirjo M PM; Sattin, Sara S; Beitel, Lenore K LK; Roldan, Ariel A; Colombo, Giorgio G; Balch, William W; Young, Jason C JC; Lukacs, Gergely L GL

Publication Date: 2017-08-30

Variant appearance in text: CFTR: Q637R

PubMed Link: 28855508

Variant Present in the following documents:

• 41467_2017_444_MOESM1_ESM.pdf

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Development and characterization of synthetic antibodies binding to the cystic fibrosis conductance regulator.

Mabs

Gakhal, Amandeep K AK; Jensen, Timothy J TJ; Bozoky, Zoltan Z; Roldan, Ariel A; Lukacs, Gergely L GL; Forman-Kay, Julie J; Riordan, John R JR; Sidhu, Sachdev S SS

Publication Date: 2016

Variant appearance in text: CFTR: Q637R

PubMed Link: 27185291

Variant Present in the following documents:

• Main text

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Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.

The Journal Of Biological Chemistry

Gong, Xiaoyan X; Ahner, Annette A; Roldan, Ariel A; Lukacs, Gergely L GL; Thibodeau, Patrick H PH; Frizzell, Raymond A RA

Publication Date: 2016-01-22

Variant appearance in text: CFTR: Q637R

PubMed Link: 26627832

Variant Present in the following documents:

• Main text

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Binding screen for cystic fibrosis transmembrane conductance regulator correctors finds new chemical matter and yields insights into cystic fibrosis therapeutic strategy.

Protein Science : A Publication Of The Protein Society

Hall, Justin D JD; Wang, Hong H; Byrnes, Laura J LJ; Shanker, Suman S; Wang, Kelong K; Efremov, Ivan V IV; Chong, P Andrew PA; Forman-Kay, Julie D JD; Aulabaugh, Ann E AE

Publication Date: 2016-02

Variant appearance in text: CFTR: Q637R

PubMed Link: 26444971

Variant Present in the following documents:

• Main text

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Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assembly.

Journal Of Molecular Biology

He, Lihua L; Aleksandrov, Andrei A AA; An, Jianli J; Cui, Liying L; Yang, Zhengrong Z; Brouillette, Christie G CG; Riordan, John R JR

Publication Date: 2015-01-16

Variant appearance in text: CFTR: Q637R

PubMed Link: 25083918

Variant Present in the following documents:

• Main text

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Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.

Molecular Pharmacology

Phuan, Puay-Wah PW; Veit, Guido G; Tan, Joseph J; Roldan, Ariel A; Finkbeiner, Walter E WE; Lukacs, Gergely L GL; Verkman, A S AS

Publication Date: 2014-07

Variant appearance in text: CFTR: Q637R

PubMed Link: 24737137

Variant Present in the following documents:

• Main text

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Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.

The Journal Of Physiology

Xu, Zhe Z; Pissarra, Luísa S LS; Farinha, Carlos M CM; Liu, Jia J; Cai, Zhiwei Z; Thibodeau, Patrick H PH; Amaral, Margarida D MD; Sheppard, David N DN

Publication Date: 2014-05-01

Variant appearance in text: CFTR: Q637R

PubMed Link: 24591578

Variant Present in the following documents:

• Main text

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Allosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTR.

Plos One

Dawson, Jennifer E JE; Farber, Patrick J PJ; Forman-Kay, Julie D JD

Publication Date: 2013

Variant appearance in text: CFTR: Q637R

PubMed Link: 24058550

Variant Present in the following documents:

• Main text

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VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.

Molecular Biology Of The Cell

Ren, Hong Yu HY; Grove, Diane E DE; De La Rosa, Oxana O; Houck, Scott A SA; Sopha, Pattarawut P; Van Goor, Fredrick F; Hoffman, Beth J BJ; Cyr, Douglas M DM

Publication Date: 2013-10

Variant appearance in text: CFTR: Q637R

PubMed Link: 23924900

Variant Present in the following documents:

• Main text

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Mechanism-based corrector combination restores ΔF508-CFTR folding and function.

Nature Chemical Biology

Okiyoneda, Tsukasa T; Veit, Guido G; Dekkers, Johanna F JF; Bagdany, Miklos M; Soya, Naoto N; Xu, Haijin H; Roldan, Ariel A; Verkman, Alan S AS; Kurth, Mark M; Simon, Agnes A; Hegedus, Tamas T; Beekman, Jeffrey M JM; Lukacs, Gergely L GL

Publication Date: 2013-07

Variant appearance in text: CFTR: Q637R

PubMed Link: 23666117

Variant Present in the following documents:

• Main text

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Cystic fibrosis transmembrane conductance regulator (ABCC7) structure.

Cold Spring Harbor Perspectives In Medicine

Hunt, John F JF; Wang, Chi C; Ford, Robert C RC

Publication Date: 2013-02-01

Variant appearance in text: CFTR: Q637R

PubMed Link: 23378596

Variant Present in the following documents:

• Main text

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Functional Rescue of F508del-CFTR Using Small Molecule Correctors.

Frontiers In Pharmacology

Molinski, Steven S; Eckford, Paul D W PD; Pasyk, Stan S; Ahmadi, Saumel S; Chin, Stephanie S; Bear, Christine E CE

Publication Date: 2012

Variant appearance in text: CFTR: Q637R

PubMed Link: 23055971

Variant Present in the following documents:

• Main text
• fphar-03-00160.pdf

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Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect.

Human Molecular Genetics

Colas, Julien J; Faure, Grazyna G; Saussereau, Emilie E; Trudel, Stéphanie S; Rabeh, Wael M WM; Bitam, Sara S; Guerrera, Ida Chiara IC; Fritsch, Janine J; Sermet-Gaudelus, Isabelle I; Davezac, Noëlie N; Brouillard, Franck F; Lukacs, Gergely L GL; Herrmann, Harald H; Ollero, Mario M; Edelman, Aleksander A

Publication Date: 2012-02-01

Variant appearance in text: CFTR: Q637R

PubMed Link: 22038833

Variant Present in the following documents:

• Main text

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The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR.

The Journal Of Physiology

Jih, Kang-Yang KY; Li, Min M; Hwang, Tzyh-Chang TC; Bompadre, Silvia G SG

Publication Date: 2011-06-01

Variant appearance in text: CFTR: Q637R

PubMed Link: 21486785

Variant Present in the following documents:

• Main text

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Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.

Protein Science : A Publication Of The Protein Society

Wang, Chi C; Protasevich, Irina I; Yang, Zhengrong Z; Seehausen, Derek D; Skalak, Timothy T; Zhao, Xun X; Atwell, Shane S; Spencer Emtage, J J; Wetmore, Diana R DR; Brouillette, Christie G CG; Hunt, John F JF

Publication Date: 2010-10

Variant appearance in text: CFTR: Q637R

PubMed Link: 20687163

Variant Present in the following documents:

• Main text

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Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1.

Protein Science : A Publication Of The Protein Society

Protasevich, Irina I; Yang, Zhengrong Z; Wang, Chi C; Atwell, Shane S; Zhao, Xun X; Emtage, Spencer S; Wetmore, Diana D; Hunt, John F JF; Brouillette, Christie G CG

Publication Date: 2010-10

Variant appearance in text: CFTR: Q637R

PubMed Link: 20687133

Variant Present in the following documents:

• Main text

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Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins.

The Journal Of Biological Chemistry

Da Paula, Ana Carina AC; Sousa, Marisa M; Xu, Zhe Z; Dawson, Elizabeth S ES; Boyd, A Christopher AC; Sheppard, David N DN; Amaral, Margarida D MD

Publication Date: 2010-08-27

Variant appearance in text: CFTR: Q637R

PubMed Link: 20551307

Variant Present in the following documents:

• Main text

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NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.

The Embo Journal

Kanelis, Voula V; Hudson, Rhea P RP; Thibodeau, Patrick H PH; Thomas, Philip J PJ; Forman-Kay, Julie D JD

Publication Date: 2010-01-06

Variant appearance in text: CFTR: Q637R

PubMed Link: 19927121

Variant Present in the following documents:

• Main text

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Cooperative assembly and misfolding of CFTR domains in vivo.

Molecular Biology Of The Cell

Du, Kai K; Lukacs, Gergely L GL

Publication Date: 2009-04

Variant appearance in text: CFTR: Q637R

PubMed Link: 19176754

Variant Present in the following documents:

• Main text

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