Publications:

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A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins.

Scientific Reports

Shishido, Hideki H; Yoon, Jae Seok JS; Skach, William R WR

Publication Date: 2022-02-15

Variant appearance in text: CFTR: S492P

PubMed Link: 35169219

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• 41598_2022_Article_6456.pdf

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A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins.

Scientific Reports

Shishido, Hideki H; Yoon, Jae Seok JS; Skach, William R WR

Publication Date: 2022-02-15

Variant appearance in text: CFTR: S492P

PubMed Link: 35169219

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• Main text
• 41598_2022_Article_6456.pdf

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CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.

Nature Communications

Shishido, Hideki H; Yoon, Jae Seok JS; Yang, Zhongying Z; Skach, William R WR

Publication Date: 2020-08-26

Variant appearance in text: CFTR: S492P

PubMed Link: 32848127

Variant Present in the following documents:

• Main text
• 41467_2020_Article_18101.pdf
• 41467_2020_18101_MOESM1_ESM.pdf

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Disease-relevant mutations alter amino acid co-evolution networks in the second nucleotide binding domain of CFTR.

Plos One

Ivey, Gabrianne G; Youker, Robert T RT

Publication Date: 2020

Variant appearance in text: CFTR: S492P

PubMed Link: 31978131

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• pone.0227668.pdf

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Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.

Nature Communications

Sigoillot, Maud M; Overtus, Marie M; Grodecka, Magdalena M; Scholl, Daniel D; Garcia-Pino, Abel A; Laeremans, Toon T; He, Lihua L; Pardon, Els E; Hildebrandt, Ellen E; Urbatsch, Ina I; Steyaert, Jan J; Riordan, John R JR; Govaerts, Cedric C

Publication Date: 2019-06-14

Variant appearance in text: CFTR: S492P

PubMed Link: 31201318

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• Main text
• 41467_2019_Article_10714.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: S492P

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: S492P

PubMed Link: 29425673

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Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.

Protein Engineering, Design & Selection : Peds

Xavier, B M BM; Hildebrandt, E E; Jiang, F F; Ding, H H; Kappes, J C JC; Urbatsch, I L IL

Publication Date: 2017-10-01

Variant appearance in text: CFTR: S492P

PubMed Link: 29053845

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Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.

The Journal Of Biological Chemistry

Vernon, Robert M RM; Chong, P Andrew PA; Lin, Hong H; Yang, Zhengrong Z; Zhou, Qingxian Q; Aleksandrov, Andrei A AA; Dawson, Jennifer E JE; Riordan, John R JR; Brouillette, Christie G CG; Thibodeau, Patrick H PH; Forman-Kay, Julie D JD

Publication Date: 2017-08-25

Variant appearance in text: CFTR: S492P

PubMed Link: 28655774

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Specific stabilization of CFTR by phosphatidylserine.

Biochimica Et Biophysica Acta. Biomembranes

Hildebrandt, Ellen E; Khazanov, Netaly N; Kappes, John C JC; Dai, Qun Q; Senderowitz, Hanoch H; Urbatsch, Ina L IL

Publication Date: 2017-02

Variant appearance in text: CFTR: S492P

PubMed Link: 27913277

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Thermal stability of purified and reconstituted CFTR in a locked open channel conformation.

Protein Expression And Purification

Aleksandrov, Luba A LA; Jensen, Timothy J TJ; Cui, Liying L; Kousouros, Joseph N JN; He, Lihua L; Aleksandrov, Andrei A AA; Riordan, John R JR

Publication Date: 2015-12

Variant appearance in text: CFTR: S492P

PubMed Link: 26384709

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Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.

The Journal Of Biological Chemistry

Chong, P Andrew PA; Farber, Patrick J PJ; Vernon, Robert M RM; Hudson, Rhea P RP; Mittermaier, Anthony K AK; Forman-Kay, Julie D JD

Publication Date: 2015-09-18

Variant appearance in text: CFTR: S492P

PubMed Link: 26149808

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Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.

The Journal Of Physiology

Cai, Zhiwei Z; Palmai-Pallag, Timea T; Khuituan, Pissared P; Mutolo, Michael J MJ; Boinot, Clément C; Liu, Beihui B; Scott-Ward, Toby S TS; Callebaut, Isabelle I; Harris, Ann A; Sheppard, David N DN

Publication Date: 2015-06-01

Variant appearance in text: CFTR: S492P

PubMed Link: 25763566

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Rational Coupled Dynamics Network Manipulation Rescues Disease-Relevant Mutant Cystic Fibrosis Transmembrane Conductance Regulator.

Chemical Science

Proctor, Elizabeth A EA; Kota, Pradeep P; Aleksandrov, Andrei A AA; He, Lihua L; Riordan, John R JR; Dokholyan, Nikolay V NV

Publication Date: 2015-02

Variant appearance in text: CFTR: S492P

PubMed Link: 25685315

Variant Present in the following documents:

• Main text
• SC-006-C4SC01320D-s001.pdf
• SC-006-C4SC01320D.pdf

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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: S492P

PubMed Link: 25083918

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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: S492P

PubMed Link: 24591578

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Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein.

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

He, Lihua L; Kota, Pradeep P; Aleksandrov, Andrei A AA; Cui, Liying L; Jensen, Tim T; Dokholyan, Nikolay V NV; Riordan, John R JR

Publication Date: 2013-02

Variant appearance in text: CFTR: S492P

PubMed Link: 23104983

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Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.

Journal Of Molecular Biology

Aleksandrov, Andrei A AA; Kota, Pradeep P; Cui, Liying L; Jensen, Tim T; Alekseev, Alexey E AE; Reyes, Santiago S; He, Lihua L; Gentzsch, Martina M; Aleksandrov, Luba A LA; Dokholyan, Nikolay V NV; Riordan, John R JR

Publication Date: 2012-05-25

Variant appearance in text: CFTR: S492P

PubMed Link: 22406676

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