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Article

Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.

Proceedings of the National Academy of Sciences of the United States of America - 25 May 2004

Cormet-Boyaka Estelle, Jablonsky Michael, Naren Anjaparavanda P, Jackson Patricia L, Muccio Donald D, Kirk Kevin L

Abstract excerpt

Most cases of cystic fibrosis (CF) are caused by mutations that block the biosynthetic maturation of the CF gene product, the CF transmembrane conductance regulator (CFTR) chloride channel. CFTR-processing mutants fail to escape the endoplasmic reticulum and are rapidly degraded. Current efforts to induce the maturation of CFTR mutants target components of the biosynthetic pathway (e.g., chaperones) rather than...

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Source

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Identifiers

PMID: 15141088
DOI: 10.1073/pnas.0400459101
PMCID: PMC419584

Genes

CFTR

Variants

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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Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.PMID 15141088Read ↗

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Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.Semantic proximity Read ↗

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