Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations.

American journal of physiology. Lung cellular and molecular physiology - 1 Nov 2020

Wang Wei, Fu Lianwu, Liu Zhiyong, Wen Hui, Rab Andras, Hong Jeong S, Kirk Kevin L, Rowe Steven M

Abstract excerpt

G551D is a major disease-associated gating mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP- and phosphorylation-dependent chloride channel. G551D causes severe cystic fibrosis (CF) disease by disrupting ATP-dependent channel opening; however, whether G551D affects phosphorylation-dependent channel activation is unclear. Here, we use macropatch recording and Ussing...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Adenosine Triphosphate
Share to Chloride Channels

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Cyclic AMP-Dependent Protein Kinases

Share to Cystic Fibrosis

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Ion Channel Gating

Share to Mutation

Share to Signal Transduction

Source

Open in PubMed Open full text

Identifiers

PMID: 32877225
DOI: 10.1152/ajplung.00262.2019
PMCID: PMC7701354

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations.PMID 32877225Read ↗

A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.Semantic proximity Read ↗

Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease.Semantic proximity Read ↗

Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.Semantic proximity Read ↗

Physiological and pharmacological characterization of the N1303K mutant CFTR.Semantic proximity Read ↗

Electrophysiological, biochemical, and bioinformatic methods for studying CFTR channel gating and its regulation.Semantic proximity Read ↗

Altered functional interactions between CFTR disease mutants ΔF508 and G551D and the protein kinase A catalytic subunit.Semantic proximity Read ↗

VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.Semantic proximity Read ↗

Influence of Disease-Causing Mutations and Ivacaftor on the Dynamics of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ProteinSemantic proximity Read ↗

Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis.Semantic proximity Read ↗

Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs.Semantic proximity Read ↗

Structural determinants of protein kinase A essential for CFTR channel activationSemantic proximity Read ↗

G551D and G1349D, Two CF-associated Mutations in the Signature Sequences of CFTR, Exhibit Distinct Gating DefectsSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.