Nucleotide-binding domains of human Cystic Fibrosis Transmembrane Conductance Regulator: detailed sequence analysis and three-dimensional modeling of the heterodimer.
type: Dataset
description: The cystic fibrosis transmembrane conductance regulator (CFTR) protein is encoded by the gene that is defective in cystic fibrosis, the most common lethal inherited disease among the Caucasian population. CFTR belongs to the ABC transporter superfamily, whose members form macromolecular architectures composed of two membrane-spanning domains and two nucleotide-binding domains (NBDs). The experimental structures of NBDs from several ABC transporters have recently been solved, opening new avenues for understanding the structure/function relationships and the consequences of some disease-causing mutations of CFTR. Based on a detailed sequence/structure analysis, we propose here a three-dimensional model of the human CFTR NBD heterodimer. This model, which is in agreement with recent experimental data, highlights the specific features of the CFTR asymmetric active sites located at the interface between the two NBDs. Moreover, additional CFTR-specific features can be identified at the subunit interface, which may play critical roles in active site interdependence and are uncommon in other NBD dimers.
landing page: 1420-682X
keyword: abc-transporter
keyword: cftr
keyword: cystic-fibrosis
keyword: disease-causing-mutation
keyword: hydrophobic-cluster-analysis
keyword: infoeu-reposemanticsarticle
keyword: journal-articles
keyword: molecular-modeling
keyword: mrp1
keyword: sur1
issued: 2026-05-09T20:26:12.545498
modified: 2026-05-09T20:26:12.545502
language: en
contact point: Organization
  fn: CCSD

test_moissonnage_selune
type: Agent

Nucleotide-binding domains of human Cystic Fibrosis Transmembrane Conductance Regulator: detailed sequence analysis and three-dimensional modeling of the heterodimer.
type: Distribution
format: HTML
access URL: https://hal.science/hal-00085903
issued: 2026-05-09T20:26:12.546982
modified: 2026-05-09T20:26:12.532425