David N Sheppard
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Explore the profile of David N Sheppard including associated specialties, affiliations and a list of published articles.
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80
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1844
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Recent Articles
1.
Vila-Gonzalez M, Pinte L, Fradique R, Causa E, Kool H, Rodrat M, et al.
Respir Res
. 2024 Apr;
25(1):180.
PMID: 38664797
Background: Pulmonary ionocytes have been identified in the airway epithelium as a small population of ion transporting cells expressing high levels of CFTR (cystic fibrosis transmembrane conductance regulator), the gene...
2.
Li H, Rodrat M, Al-Salmani M, Veselu D, Han S, Raraigh K, et al.
J Physiol
. 2024 Jan;
602(2):333-354.
PMID: 38186087
Some residues in the cystic fibrosis transmembrane conductance regulator (CFTR) channel are the site of more than one CFTR variant that cause cystic fibrosis. Here, we investigated the function of...
3.
Urbaniak A, Thummel K, Alade A, Rettie A, Prasad B, De Nicolo A, et al.
Pharmacol Res Perspect
. 2023 Oct;
11(6):e01147.
PMID: 37885364
No abstract available.
4.
Yeh H, Sutcliffe K, Sheppard D, Hwang T
Handb Exp Pharmacol
. 2022 Aug;
283:219-247.
PMID: 35972584
People with cystic fibrosis (CF) suffer from a multi-organ disorder caused by loss-of-function variants in the gene encoding the epithelial anion channel cystic fibrosis transmembrane conductance regulator (CFTR). Tremendous progress...
5.
Kelly J, Al-Rammahi M, Daly K, Flanagan P, Urs A, Cohen M, et al.
Sci Rep
. 2022 Apr;
12(1):6593.
PMID: 35449374
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Defective CFTR leads to accumulation of dehydrated viscous mucus within the small intestine, luminal...
6.
Raraigh K, Paul K, Goralski J, Worthington E, Faino A, Sciortino S, et al.
JCI Insight
. 2022 Mar;
7(6).
PMID: 35315358
The chloride channel dysfunction caused by deleterious cystic fibrosis transmembrane conductance regulator (CFTR) variants generally correlates with severity of cystic fibrosis (CF). However, 3 adults bearing the common severe variant...
7.
Prins S, Corradi V, Sheppard D, Tieleman D, Vergani P
J Biol Chem
. 2022 Jan;
298(3):101615.
PMID: 35065958
Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most common cause of cystic fibrosis. The F508 residue is located on nucleotide-binding...
8.
Noel S, Servel N, Hatton A, Golec A, Rodrat M, Ng D, et al.
J Physiol
. 2021 Nov;
600(6):1515-1531.
PMID: 34761808
Dysfunction of the epithelial anion channel cystic fibrosis transmembrane conductance regulator (CFTR) causes a wide spectrum of disease, including cystic fibrosis (CF) and CFTR-related diseases (CFTR-RDs). Here, we investigate genotype-phenotype-CFTR...
9.
Liu J, Berg A, Wang Y, Jantarajit W, Sutcliffe K, Stevens E, et al.
Br J Pharmacol
. 2021 Oct;
179(7):1319-1337.
PMID: 34644413
Background And Purpose: Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators are small molecules developed to treat the genetic disease cystic fibrosis (CF). They interact directly with CFTR Cl channels at...
10.
Scholl D, Sigoillot M, Overtus M, Martinez R, Martens C, Wang Y, et al.
Nat Chem Biol
. 2021 Aug;
17(9):989-997.
PMID: 34341587
The cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is essential to maintain fluid homeostasis in key organs. Functional impairment of CFTR due to mutations in the cftr gene leads...