Epithelial IgG and its relationship to the loss of F508 in the common mutant form of the cystic fibrosis transmembrane conductance regulator

The most debilitating feature of cystic fibrosis (CF) disease is uncontrolled inflammation of respiratory epithelium. The relationship between the commonest mutated form of CFTR (F508del or ΔF508) and inflammation has not yet been elucidated. Here, we present a new paradigm suggesting that CFTR can...

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Veröffentlicht in:FEBS letters 2009-08, Vol.583 (15), p.2493-2499
Hauptverfasser: Treharne, Kate J., Cassidy, Diane, Goddard, Catharine, Colledge, William H., Cassidy, Andrew, Mehta, Anil
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container_end_page 2499
container_issue 15
container_start_page 2493
container_title FEBS letters
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creator Treharne, Kate J.
Cassidy, Diane
Goddard, Catharine
Colledge, William H.
Cassidy, Andrew
Mehta, Anil
description The most debilitating feature of cystic fibrosis (CF) disease is uncontrolled inflammation of respiratory epithelium. The relationship between the commonest mutated form of CFTR (F508del or ΔF508) and inflammation has not yet been elucidated. Here, we present a new paradigm suggesting that CFTR can interact with intra-epithelial IgG, establishing a direct link between normal CFTR and the immune system. Further, our data show that the amino-acid sequence local to F508 can bind IgG with high affinity, dependent on F508, such that loss of F508 abolishes this link both in vitro and in the intact cell.
doi_str_mv 10.1016/j.febslet.2009.07.002
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source Wiley Free Content; MEDLINE; Wiley Online Library Journals Frontfile Complete; Elsevier ScienceDirect Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection
subjects Amino Acid Sequence
Animals
Antigen presentation/processing
Cell surface molecules
Cystic Fibrosis - immunology
Cystic Fibrosis - pathology
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Cystic Fibrosis Transmembrane Conductance Regulator - metabolism
Epithelial Cells - cytology
Epithelial Cells - immunology
Epithelial Cells - physiology
Humans
Immunoglobulin G - metabolism
Inflammation
Mice
Mice, Transgenic
Molecular Sequence Data
Mucosa
Mutation
Peptides - genetics
Peptides - metabolism
Phenylalanine - metabolism
Poxviridae - genetics
Poxviridae - metabolism
Respiratory Mucosa - cytology
Respiratory Mucosa - immunology
Respiratory Mucosa - pathology
Sequence Alignment
Sheep
Smallpox
title Epithelial IgG and its relationship to the loss of F508 in the common mutant form of the cystic fibrosis transmembrane conductance regulator
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