D. Sheppard

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Recommendations for the classification of diseases as CFTR-related disorders.

B. Kerem, J. Zieleński, P. Pignatti, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Alteration of protein function by a silent polymorphism linked to tRNA abundance

Z. Ignatova, Robert Rauscher, I. Braakman, 2017, PLoS biology.

Transepithelial electrical measurements with the Ussing chamber.

D. Sheppard, Hongyu Li, M. Hug, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

The therapeutic potential of small-molecule modulators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel

S. Husbands, D. Sheppard, P. Khuituan, 2014 .

Function of CFTR Protein: Ion Transport

D. Sheppard, Z. Cai, Hongyu Li, 2006 .

Mouse models of cystic fibrosis: phenotypic analysis and research applications.

L. Touqui, W. Colledge, M. Wilke, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR☆

P. Haggie, D. Sheppard, M. Schlierf, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Potentiation of the cystic fibrosis transmembrane conductance regulator Cl- channel by ivacaftor is temperature independent.

D. Sheppard, Z. Cai, M. Gosling, 2018, American journal of physiology. Lung cellular and molecular physiology.

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

I. Callebaut, D. Sheppard, P. Khuituan, 2015, The Journal of physiology.

Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms

C. Soares, M. Amaral, D. Sheppard, 2006, Proceedings of the National Academy of Sciences.

Partial rescue of F508del‐cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual‐acting small molecule

Nikhil T. Awatade, M. Amaral, C. Farinha, 2018, British journal of pharmacology.

Potassium currents of isolated Necturus enterocytes: a whole‐cell patch‐clamp study.

M. Valverde, D. Sheppard, F. Giráldez, 1991, The Journal of physiology.

Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors

D. Sheppard, Yiting Wang, M. Gray, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Loop diuretics are open‐channel blockers of the cystic fibrosis transmembrane conductance regulator with distinct kinetics

S. Husbands, D. Sheppard, P. Khuituan, 2014, British journal of pharmacology.

New clinical diagnostic procedures for cystic fibrosis in Europe.

N. Derichs, F. Vermeulen, I. Fajac, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Murine epithelial cells: isolation and culture.

S. Randell, D. Davidson, R. Tarran, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

A chloride conductance activated by adenosine 3',5'‐cyclic monophosphate in the apical membrane of Necturus enterocytes.

D. Sheppard, F. Giráldez, F. Sepúlveda, 1988, The Journal of physiology.

CpG-free plasmids confer reduced inflammation and sustained pulmonary gene expression

David N Sheppard, Uta Griesenbach, David J Porteous, 2008, Nature Biotechnology.

Inhibition of Protein Kinase CK2 Closes the CFTR Cl- Channel, but has no Effect on the Cystic Fibrosis Mutant ΔF508-CFTR

A. Mehta, P. Hegyi, O. G. Best, 2009, Cellular Physiology and Biochemistry.

Phosphate stimulates CFTR Cl- channels.

M. Welsh, D. Sheppard, M. R. Carson, 1994, Biophysical journal.

Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site.

A. Mehta, K. Kunzelmann, D. Gruenert, 2007, The Journal of biological chemistry.

Parathyroid hormone increases CFTR expression and function in Caco-2 intestinal epithelial cells.

D. Sheppard, N. Charoenphandhu, Kannikar Wongdee, 2020, Biochemical and biophysical research communications.

Inhibition of Protein Kinase CK 2 Closes the CFTR Cl-Channel , but has no Effect on the Cystic Fibrosis Mutant Δ F 508-CFTR

A. Mehta, P. Hegyi, K. Kunzelmann, 2009 .

Targeted anion transporter delivery by coiled-coil driven membrane fusion† †Electronic supplementary information (ESI) available. See DOI: 10.1039/c5sc04282h Click here for additional data file.

T. Sharp, A. Kros, D. Sheppard, 2016, Chemical science.

A primary culture model of differentiated murine tracheal epithelium.

S. Randell, D. Davidson, J. Dorin, 2000, American journal of physiology. Lung cellular and molecular physiology.

Residual function of cystic fibrosis mutants predicts response to small molecule CFTR modulators.

G. Cutting, A. Joynt, T. Evans, 2018, JCI insight.

Chloride transport across vesicle and cell membranes by steroid-based receptors.

Bradley D. Smith, T. Lambert, A. Koulov, 2003, Angewandte Chemie.

Development of synthetic membrane transporters for anions.

David N Sheppard, Bradley D. Smith, Bradley D Smith, 2007, Chemical Society reviews.

Nonprotonophoric Electrogenic Cl− Transport Mediated by Valinomycin-like Carriers

Philip A. Gale, E. Howe, V. Soto-Cerrato, 2016 .

Efficient, non-toxic anion transport by synthetic carriers in cells and epithelia

S. Hussain, D. Sheppard, A. P. Davis, 2015, Nature Chemistry.

Preorganized bis-thioureas as powerful anion carriers: chloride transport by single molecules in large unilamellar vesicles.

S. Hussain, D. Sheppard, A. P. Davis, 2014, Journal of the American Chemical Society.

Structure and function of the CFTR chloride channel.

M. Welsh, D. Sheppard, M J Welsh, 1999, Physiological reviews.

Mutations in CFTR associated with mild-disease-form CI- channels with altered pore properties

M. Welsh, Alan E. Smith, D. Sheppard, 1993, Nature.

Anion transport by ortho-phenylene bis-ureas across cell and vesicle membranes.

Philip A. Gale, D. Sheppard, A. P. Davis, 2018, Organic & biomolecular chemistry.

Therapeutic approaches to CFTR dysfunction: From discovery to drug development.

N. Pedemonte, Ashvani K. Singh, D. Sheppard, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Bypassing CFTR dysfunction in cystic fibrosis with alternative pathways for anion transport

D. Sheppard, Hongyu Li, M. Mall, 2017, Current opinion in pharmacology.

Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.

M. Welsh, G. Denning, D. Sheppard, 1994, The American journal of physiology.

Cystic fibrosis transmembrane conductance regulator: A chloride channel with novel regulation

Matthew P. Anderson, H. A. Berger, M. Welsh, 1992, Neuron.

The CFTR Cl- channel: a versatile engine for transepithelial ion transport

D. Sheppard, Z. Cai, M. Ju, 2007 .

The cystic fibrosis transmembrane conductance regulator Cl⁻ channel: a versatile engine for transepithelial ion transport.

D. Sheppard, Z. Cai, M. Ju, 2007, Sheng li xue bao : [Acta physiologica Sinica].

Mechanism of Glibenclamide Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channels Expressed in a Murine Cell Line

D. Sheppard, K. Robinson, 1997, The Journal of physiology.

A small molecule CFTR potentiator restores ATP‐dependent channel gating to the cystic fibrosis mutant G551D‐CFTR

Lishuang Cao, E. Stevens, D. Sheppard, 2021, British journal of pharmacology.

Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl− channels expressed in mammalian cells

D. Sheppard, B. Wainwright, K. Lansdell, 1998, The Journal of physiology.

Molecular pharmacology of the CFTR Cl- channel.

D. Sheppard, T. Hwang, David N. Sheppard, 1999, Trends in pharmacological sciences.

Understanding how cystic fibrosis mutations cause a loss of Cl- channel function.

D. Sheppard, L. Ostedgaard, 1996, Molecular medicine today.

CFTR: New insights into structure and function and implications for modulation by small molecules.

J. Hunt, I. Sermet-Gaudelus, I. Callebaut, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

Garry R. Cutting, Harvey B. Pollard, Jeffrey L. Brodsky, 2016, Molecular biology of the cell.

The amino-terminal portion of CFTR forms a regulated CI− channel

M. Welsh, D. Sheppard, L. Ostedgaard, 1994, Cell.

Gating of the CFTR Cl− channel by ATP‐driven nucleotide‐binding domain dimerisation

D. Sheppard, T. Hwang, 2009, The Journal of physiology.

Differential Sensitivity of the Cystic Fibrosis (CF)-associated Mutants G551D and G1349D to Potentiators of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl– Channel*

A. Taddei, D. Sheppard, Z. Cai, 2006, Journal of Biological Chemistry.

Chapter 5 - The Physiology and Pharmacology of the CFTR Cl− Channel

D. Sheppard, Z. Cai, Hongyu Li, 2006 .

Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.

H. A. Berger, M. Welsh, D. Sheppard, 1992, The American journal of physiology.

Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains

D. Tieleman, V. Corradi, D. Sheppard, 2021, bioRxiv.

Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR

D. Sheppard, Z. Cai, H. D. de Jonge, 2019, American journal of physiology. Lung cellular and molecular physiology.

Sheppard, D. (2019). Differential thermostability and response to cystic fibrosis transmembrane conductance regulator (CFTR) potentiators of human and mouse F508del-CFTR. AJP-Lung Cellular and Molecular Physiology, 317(1), L71-L86. https://doi.org/10.1152/ajplung.00034.2019

D. Sheppard, Z. Cai, Jia Liu, 2019 .

Murine CFTR Channel and its Role in Regulatory Volume Decrease of Small Intestine Crypts

W. Colledge, M. Valverde, B. Wainwright, 2001, Cellular Physiology and Biochemistry.

Function of Xenopus Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl− Channels and Use of Human-Xenopus Chimeras to Investigate the Pore Properties of CFTR*

M. Welsh, D. Sheppard, H. Ishihara, 1996, The Journal of Biological Chemistry.

The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.

David N Sheppard, D. Sheppard, Hongyu Li, 2004, Kidney international.

Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents

M. Welsh, D. Sheppard, David N. Sheppard, 1992, The Journal of general physiology.

The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel.

D. Benos, P. Linsdell, C. Bear, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.

M. Amaral, D. Sheppard, Hongyu Li, 2012, American journal of physiology. Renal physiology.

A topological switch in CFTR modulates channel activity and sensitivity to unfolding

E. Pardon, J. Steyaert, A. Garcia-Pino, 2021, Nature Chemical Biology.

Therapeutic Potential of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitors in Polycystic Kidney Disease

D. Sheppard, Hongyu Li, 2012, BioDrugs.

Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants.

D. Sheppard, Y. Sohma, Z. Cai, 2011, Methods in molecular biology.

Strategies to investigate the mechanism of action of CFTR modulators.

D. Sheppard, Z. Cai, Hongyu Li, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Voltage-dependent Gating of the Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel

D. Sheppard, Z. Cai, T. Scott-Ward, 2003, The Journal of general physiology.

Phloxine B Interacts with the Cystic Fibrosis Transmembrane Conductance Regulator at Multiple Sites to Modulate Channel Activity* 210

D. Sheppard, Z. Cai, 2002, The Journal of Biological Chemistry.

Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods.

J. Riordan, R. Dérand, G. Heda, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Chapter 7 The CFTR Chloride Channel

Matthew P. Anderson, H. A. Berger, M. Welsh, 1994 .

Pharmacological therapy for cystic fibrosis: from bench to bedside.

M. Conese, D. Sheppard, M. Mall, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Section D (cell physiology): an armamentarium to investigate CFTR function

D. Sheppard, M. Hug, 2004 .

CFTR potentiators partially restore channel function to A561E‐CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del‐CFTR

A. Toye, D. Sheppard, Z. Cai, 2014, British journal of pharmacology.

Correlating genotype with phenotype using CFTR‐mediated whole‐cell Cl− currents in human nasal epithelial cells

I. Sermet-Gaudelus, N. Servel, D. Sheppard, 2021, The Journal of physiology.

Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis

D. Sheppard, Z. Cai, Hong-yu Li, 2011, Acta Pharmacologica Sinica.

Direct Sensing of Intracellular pH by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl− Channel*♦

D. Sheppard, Z. Cai, Jeng-Haur Chen, 2009, The Journal of Biological Chemistry.

Understanding how cystic fibrosis mutations disrupt CFTR function: from single molecules to animal models.

D. Sheppard, Z. Cai, Hongyu Li, 2014, The international journal of biochemistry & cell biology.

CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor

C. Merlo, S. Sciortino, H. Ling, 2022, JCI insight.

Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.

M. Welsh, D. Sheppard, L. Ostedgaard, 1995, The EMBO journal.

Chronic ivacaftor treatment: getting F508del-CFTR into more trouble?

D. Sheppard, M. Mall, 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

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

M. Amaral, C. Farinha, D. Sheppard, 2014, The Journal of physiology.

Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease

K. Daly, M. Al-Rammahi, S. Shirazi-Beechey, 2022, Scientific Reports.

Effect of ATP concentration on CFTR Cl- channels: a kinetic analysis of channel regulation.

M. Welsh, D. Sheppard, M. R. Carson, 1994, Biophysical journal.

Preferred Formation of Heteromeric Channels between Coexpressed SK1 and IKCa Channel Subunits Provides a Unique Pharmacological Profile of Ca2+-Activated Potassium Channels

N. Marrion, P. Colarusso, R. W. Turner, 2019, Molecular Pharmacology.

Acute inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel by thyroid hormones involves multiple mechanisms.

D. Sheppard, Z. Cai, Hongyu Li, 2013, American journal of physiology. Cell physiology.

Exploiting species differences to understand the CFTR Cl- channel.

D. Sheppard, Z. Cai, Samuel J. Bose, 2015, Biochemical Society transactions.

Pore-forming small molecules offer a promising way to tackle cystic fibrosis

D. Sheppard, A. Davis, 2019, Nature.

Molecular Physiology and Pharmacology of the Cystic Fibrosis Transmembrane Conductance Regulator

N. Pedemonte, Ashvani K. Singh, D. Sheppard, 2020 .

Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation*

R. Ford, D. Sheppard, Z. Cai, 2017, The Journal of Biological Chemistry.

Regulation of murine cystic fibrosis transmembrane conductance regulator Cl− channels expressed in Chinese hamster ovary cells

B. Wainwright, D. Sheppard, K. Lansdell, 1998, The Journal of physiology.

Chimeric constructs endow the human CFTR Cl− channel with the gating behavior of murine CFTR

D. Porteous, M. Amaral, A. Doherty, 2007, Proceedings of the National Academy of Sciences.

A Topological Switch in the Cystic Fibrosis Transmembrane Conductance Regulator Modulates Channel Activity and Sensitivity to Disease-Causing Mutation

E. Pardon, J. Steyaert, A. Garcia-Pino, 2020 .

A Topological Switch Enables Misfolding of the Cystic Fibrosis Transmembrane Conductance Regulator

E. Pardon, J. Steyaert, A. Garcia-Pino, 2020, bioRxiv.

Stability to a Sub-population of the Cystic Fibrosis Transmembrane conductance Regulator with the Predominant Disease-causing

R. Ford, D. Sheppard, Z. Cai, 2017 .

Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation.

M. Amaral, P. Thomas, C. Farinha, 2008, Chemistry & biology.

Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl− channel

D. Sheppard, Jeng-Haur Chen, Weiyi Xu, 2017, The Journal of physiology.

The European cystic fibrosis patient registry: the power of sharing data.

D. Sheppard, 2010, Journal of Cystic Fibrosis.

Folding and Rescue of a Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Mutant Identified Using Human-Murine Chimeric Proteins*

M. Amaral, D. Sheppard, M. Sousa, 2010, The Journal of Biological Chemistry.

Carbon monoxide-releasing molecules inhibit the cystic fibrosis transmembrane conductance regulator Cl- channel.

D. Sheppard, N. Charoenphandhu, Jia Liu, 2020, American journal of physiology. Lung cellular and molecular physiology.

Potentiation of cystic fibrosis transmembrane conductance regulator (CFTR) Cl− currents by the chemical solvent tetrahydrofuran

D. Sheppard, M. Ju, L. K. Hughes, 2008, Molecular membrane biology.

CFTR Channel Pharmacology

D. Sheppard, 2004, The Journal of general physiology.

CFTR Channel Pharmacology

D. Sheppard, 2004, The Journal of general physiology.

CFTR channel pharmacology: insight from a flock of clones. Focus on "Divergent CFTR orthologs respond differently to the channel inhibitors CFTRinh-172, glibenclamide, and GlyH-101".

D. Sheppard, 2012, American Journal of Physiology - Cell Physiology.

Contribution of Proline Residues in the Membrane-spanning Domains of Cystic Fibrosis Transmembrane Conductance Regulator to Chloride Channel Function*

M. Welsh, D. Sheppard, S. M. Travis, 1996, The Journal of Biological Chemistry.

Cystic fibrosis: CFTR correctors to the rescue.

D. Sheppard, 2011, Chemistry & biology.

Transient outward currents in cochlear ganglion neurons of the chick embryo

M. Valverde, D. Sheppard, F. Giráldez, 1992, Neuroscience.

Suppressing ‘nonsense’ in cystic fibrosis

I. Sermet-Gaudelus, D. Sheppard, A. Hinzpeter, 2019, Journal of Physiology.

Kinetics of voltage- and Ca2+ activation and Ba2+ blockade of a large-conductance K+ channel fromNecturus enterocytes

D. Sheppard, F. Giráldez, F. Sepúlveda, 1988, The Journal of Membrane Biology.

Inhibition of the Cystic Fibrosis Transmembrane Conductance Regulator By ATP‐Sensitive K+ Channel Regulators a

M. Welsh, D. Sheppard, 1993, Annals of the New York Academy of Sciences.

The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis.

N. Kaminski, D. Sheppard, D. Rifkin, 1999, Cell.

Editorial overview: Respiratory: Transformational therapies for cystic fibrosis.

C. Bear, D. Sheppard, H. D. de Jonge, 2017, Current opinion in pharmacology.

Investigating the CFTR Cl - Channel Using the Patch-Clamp Technique: An Overview

D. Sheppard, Z. Cai, Hongyu Li, 2003 .

Development of Na+- and K+-Currents in the cocheear ganglion of the chick embryo

M. Valverde, D. Sheppard, F. Giráldez, 1992, Neuroscience.

Erratum: A primary culture model of differentiated murine tracheal epithelium (American Journal Physiology - Lung Cellular and Molecular (2000) 279 (L766-L778))

S. Randell, D. Davidson, J. Dorin, 2001 .

Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator

M. Amaral, D. Sheppard, Z. Cai, 2008, British journal of pharmacology.

Exploiting species differences to understand the CFTR Cl- channel.

D. Sheppard, Z. Cai, Samuel J. Bose, 2015, Biochemical Society Transactions.

Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR

D. Sheppard, Z. Cai, H. D. de Jonge, 2019, American journal of physiology. Lung cellular and molecular physiology.

Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.

M. Amaral, D. Sheppard, Hongyu Li, 2012, AJP - Renal Physiology.

Preorganized bis-thioureas as powerful anion carriers: chloride transport by single molecules in large unilamellar vesicles.

S. Hussain, D. Sheppard, Hongyu Li, 2014, Journal of the American Chemical Society.

Fluorinated synthetic anion carriers: experimental and computational insights into transmembrane chloride transport

E. Howe, Xin Wu, D. Sheppard, 2018, Chemical science.

CFTR Modulators: From Mechanism to Targeted Therapeutics.

D. Sheppard, T. Hwang, H. Yeh, 2022, Handbook of experimental pharmacology.

New clinical diagnostic procedures for cystic fibrosis in Europe.

N. Derichs, F. Vermeulen, P. Melotti, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

EuroCareCF: working together to improve patient care and therapy development.

D. Sheppard, 2011, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

CFTR potentiators partially restore channel function to A561E‐CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del‐CFTR

A. Toye, D. Sheppard, Z. Cai, 2014, British Journal of Pharmacology.

Pore-forming small molecules offer a promising way to tackle cystic fibrosis

D. Sheppard, A. Davis, 2019, Nature.