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Article

Biochemical basis of the interaction between cystic fibrosis transmembrane conductance regulator and immunoglobulin-like repeats of filamin.

The Journal of biological chemistry - 28 May 2010

Smith Laura, Page Richard C, Xu Zhen, Kohli Ekta, Litman Paul, Nix Jay C, Ithychanda Sujay S, Liu Jianmin, Qin Jun, Misra Saurav, Liedtke Carole M

Abstract excerpt

Mutations in the chloride channel cystic fibrosis transmembrane regulator (CFTR) cause cystic fibrosis, a genetic disorder characterized by defects in CFTR biosynthesis, localization to the cell surface, or activation by regulatory factors. It was discovered recently that surface localization of CFTR is stabilized by an interaction between the CFTR N terminus and the multidomain cytoskeletal protein filamin. The...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 20351101
DOI: 10.1074/jbc.M109.080911
PMCID: PMC2878048

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Biochemical basis of the interaction between cystic fibrosis transmembrane conductance regulator and immunoglobulin-like repeats of filamin.PMID 20351101Read ↗

Cystic fibrosis transmembrane conductance regulator interacts with multiple immunoglobulin domains of filamin A.Semantic proximity Read ↗

Direct interaction with filamins modulates the stability and plasma membrane expression of CFTRSemantic proximity Read ↗

Molecular basis of filamin A-FilGAP interaction and its impairment in congenital disorders associated with filamin A mutations.Semantic proximity Read ↗

Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediate Regulation of Channel GatingSemantic proximity Read ↗

CFTR chloride channel regulation by an interdomain interaction.Semantic proximity Read ↗

Structure of filamin A immunoglobulin-like repeat 10 from Homo sapiens.Semantic proximity Read ↗

Crystal structure of the dimerization domain of human filamin ASemantic proximity Read ↗

Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling.Semantic proximity Read ↗

Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulator.Semantic proximity Read ↗

Domain-interface dynamics of CFTR revealed by stabilizing nanobodiesSemantic proximity Read ↗

Three-dimensional Reconstruction of Human Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Revealed an Ellipsoidal Structure with Orifices beneath the Putative Transmembrane DomainSemantic proximity Read ↗

Interhelical hydrogen bonds in the CFTR membrane domain.Semantic proximity Read ↗

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