Publications:

---

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: F429S

PubMed Link: 36845922

Variant Present in the following documents:

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

View BVdb publication page

---

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: F429S

PubMed Link: 36555865

Variant Present in the following documents:

• Main text
• ijms-23-16225.pdf

View BVdb publication page

---

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: F429S

PubMed Link: 35685375

Variant Present in the following documents:

• mmc1.pdf

View BVdb publication page

---

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: F429S

PubMed Link: 30498130

Variant Present in the following documents:

• Main text
• EMBJ-38-e100101.pdf

View BVdb publication page

---

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: F429S

PubMed Link: 30341603

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 30297908

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 28855508

Variant Present in the following documents:

• 41467_2017_444_MOESM1_ESM.pdf

View BVdb publication page

---

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: F429S

PubMed Link: 27185291

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 26627832

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 26444971

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 24737137

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 24591578

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 23924900

Variant Present in the following documents:

• Main text
• 3016.pdf

View BVdb publication page

---

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: F429S

PubMed Link: 23666117

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 23378596

Variant Present in the following documents:

• Main text

View BVdb publication page

---

Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.

Cell

Rabeh, Wael M WM; Bossard, Florian F; Xu, Haijin H; Okiyoneda, Tsukasa T; Bagdany, Miklos M; Mulvihill, Cory M CM; Du, Kai K; di Bernardo, Salvatore S; Liu, Yuhong Y; Konermann, Lars L; Roldan, Ariel A; Lukacs, Gergely L GL

Publication Date: 2012-01-20

Variant appearance in text: CFTR: F429S

PubMed Link: 22265408

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 20687163

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 20687133

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 20551307

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 19927121

Variant Present in the following documents:

• Main text

View BVdb publication page

---

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: F429S

PubMed Link: 19176754

Variant Present in the following documents:

• Main text

View BVdb publication page

---

Side chain and backbone contributions of Phe508 to CFTR folding.

Nature Structural & Molecular Biology

Thibodeau, Patrick H PH; Brautigam, Chad A CA; Machius, Mischa M; Thomas, Philip J PJ

Publication Date: 2005-01

Variant appearance in text: CFTR: F429S

PubMed Link: 15619636

Variant Present in the following documents:

• Main text

View BVdb publication page

---