Publications:

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Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.

The Journal Of Biological Chemistry

Chin, Stephanie S; Yang, Donghe D; Miles, Andrew J AJ; Eckford, Paul D W PDW; Molinski, Steven S; Wallace, B A BA; Bear, Christine E CE

Publication Date: 2017-02-03

Variant appearance in text: CFTR: V510C

PubMed Link: 28003367

Variant Present in the following documents:

• Main text
• zbc1988.pdf

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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: V510C

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: V510C

PubMed Link: 22406676

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Benzbromarone stabilizes ΔF508 CFTR at the cell surface.

Biochemistry

Loo, Tip W TW; Bartlett, M Claire MC; Clarke, David M DM

Publication Date: 2011-05-31

Variant appearance in text: CFTR: V510C

PubMed Link: 21520952

Variant Present in the following documents:

• Main text
• bi2004813.pdf
• bi2004813_si_001.pdf

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Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.

Journal Of Molecular Biology

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

Publication Date: 2010-08-13

Variant appearance in text: CFTR: V510C

PubMed Link: 20561529

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Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR.

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

He, Lihua L; Aleksandrov, Luba A LA; Cui, Liying L; Jensen, Timothy J TJ; Nesbitt, Kenneth L KL; Riordan, John R JR

Publication Date: 2010-08

Variant appearance in text: CFTR: V510C

PubMed Link: 20233947

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Processing mutations disrupt interactions between the nucleotide binding and transmembrane domains of P-glycoprotein and the cystic fibrosis transmembrane conductance regulator (CFTR).

The Journal Of Biological Chemistry

Loo, Tip W TW; Bartlett, M Claire MC; Clarke, David M DM

Publication Date: 2008-10-17

Variant appearance in text: CFTR: V510C

PubMed Link: 18708637

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Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function.

Proceedings Of The National Academy Of Sciences Of The United States Of America

Serohijos, Adrian W R AW; Hegedus, Tamás T; Aleksandrov, Andrei A AA; He, Lihua L; Cui, Liying L; Dokholyan, Nikolay V NV; Riordan, John R JR

Publication Date: 2008-03-04

Variant appearance in text: CFTR: V510C

PubMed Link: 18305154

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