19992020

Research output per year

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Personal profile

Research interests

The cystic fibrosis transmembrane conductance regulator (CFTR) is a novel member of the ATP-binding cassette (ABC) transporter superfamily that forms an anion channel with complex regulation. CFTR is predominantly located in epithelia lining ducts and tubes throughout the body, although it is also expressed in some non-epithelial tissues, most notably cardiac myocytes. In epithelia, CFTR provides a pathway for the movement of chloride (Cl-) and bicarbonate anions across the apical (lumen-facing) membrane and a key point at which to regulate the rate of transepithelial salt and water transport.

Dysfunction of the CFTR Cl- channel is associated with a wide spectrum of disease. Mutations that, in general, abolish the function of CFTR cause the genetic disease cystic fibrosis (CF), which affects multiple organ systems in the body. By contrast, some forms of male infertility, chronic pancreatitis and bronchiectasis are caused by mutations that probably preserve partial CFTR function. These conditions, termed CFTR-related diseases, affect a single organ system in the body. Increased or inappropriate activity of the CFTR Cl- channel is associated with other diseases, such as secretory diarrhoea and autosomal dominant polycystic kidney disease.

In our studies of the CFTR, we have three specific research goals:

  • To understand the relationship between the structure and function of the CFTR Cl- channel

  • To learn how CF-associated mutations cause a loss of CFTR function

  • To identify new modulators of CFTR that might prove to be of value in the treatment of disease and elucidate their mechanism of action.

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Projects

Research Output

  • 58 Article (Academic Journal)
  • 2 Chapter in a book
  • 2 Editorial (Academic Journal)
  • 1 Conference Contribution (Conference Proceeding)

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

Kleizen, B., Hunt, J., Callebaut, I., Hwang, T-C., Sermet-Gaudelus, I., Hafkemeyer, S. & Sheppard, D. N., Mar 2020, In : Journal of Cystic Fibrosis. 19, Supplement 1, p. S19-S24 6 p.

Research output: Contribution to journalArticle (Academic Journal)

  • 4 Citations (Scopus)

    Anion carriers as potential treatments for cystic fibrosis: transport in cystic fibrosis cells, and additivity to channel-targeting drugs

    Li, H., Valkenier, H., Thorne, A., Dias, C. M., Cooper, J. A., Keiffer, M., Busschaert, N., Gale, P. A., Sheppard, D. N. & Davis, A. P., 2 Oct 2019, In : Chemical Science. 42, p. 9663-9672 10 p.

    Research output: Contribution to journalArticle (Academic Journal)

    Open Access
    File
  • 6 Citations (Scopus)
    131 Downloads (Pure)

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

    Bose, S., Bijvelds, M. J. C., Wang, C., Liu, T., Cai, Z., Bot, A. G. M., de Jonge, H. R. & Sheppard, D., Jul 2019, In : AJP - Lung Cellular and Molecular Physiology. 317, 1, p. L71-L86 16 p.

    Research output: Contribution to journalArticle (Academic Journal)

    Open Access
    File
  • 3 Citations (Scopus)
    134 Downloads (Pure)

    Datasets

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

    Sheppard, D. N. (Creator), Wang, Y. (Creator), Cai, Z. (Contributor), Bijvelds, M. J. (Contributor), Bot, A. G. M. (Contributor) & Jonge, H. R. D. (Contributor), University of Bristol, 25 Jan 2019

    Dataset

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

    Sheppard, D. N. (Creator), Wang, Y. (Creator), Cai, Z. (Contributor), Bijvelds, M. J. C. (Contributor), Bot, A. G. M. (Contributor) & Jonge, H. R. D. (Contributor), University of Bristol, 16 Apr 2019

    Dataset

    Supervised Work

    Exploiting Species Differences to Understand the Function and Pharmacology of the Cystic Fibrosis Transmembrane Conductance Regulator

    Author: Bose, S., 23 Jan 2019

    Supervisor: Sheppard, D. (Supervisor)

    Student thesis: Doctoral ThesisDoctor of Philosophy (PhD)

    File