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Article

A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.

The Journal of general physiology - 1 Oct 2014

Lin Wen-Ying, Jih Kang-Yang, Hwang Tzyh-Chang

Abstract excerpt

Cystic fibrosis (CF), one of the most common lethal genetic diseases, is caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a chloride channel that, when phosphorylated, is gated by ATP. The third most common pathogenic mutation, a glycine-to-aspartate mutation at position 551 or G551D, shows a significantly decreased open probability (Po)...

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Identifiers

PMID: 25225552
DOI: 10.1085/jgp.201411247
PMCID: PMC4178940

Genes

CFTR

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

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A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.PMID 25225552Read ↗

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

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