Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Towards a rational combination therapy of cystic fibrosis: How cystamine restores the stability of mutant CFTR.

Autophagy - 1 Sept 2013

Villella Valeria R, Esposito Speranza, Maiuri Maria Chiara, Raia Valeria, Kroemer Guido, Maiuri Luigi

Abstract excerpt

Cystic fibrosis (CF) is most frequently due to homozygous ΔF508-CFTR mutation. The ΔF508-CFTR protein is unstable in the plasma membrane (PM), even if it is rescued by pharmacological agents that prevent its intracellular retention and degradation. Restoring defective autophagy in CF airways by proteostasis regulators (such as cystamine and its reduced form, cysteamine) can rescue and stabilize ΔF508-CFTR at the...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Cystic Fibrosis
Share to Cystic Fibrosis Transmembrane Conductance Regulator

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Drug Therapy, Combination

Share to SQSTM1 gene

Share to Models, Biological

Share to Mutant Proteins

Share to Mutation

Source

Identifiers

PMID: 23800975
DOI: 10.4161/auto.25517

Genes

CFTR
SQSTM1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Towards a rational combination therapy of cystic fibrosis: How cystamine restores the stability of mutant CFTR.PMID 23800975Read ↗

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation.Semantic proximity Read ↗

Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator.Semantic proximity Read ↗

Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutationSemantic proximity Read ↗

A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR.Semantic proximity Read ↗

Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung DiseaseSemantic proximity Read ↗

Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease.Semantic proximity Read ↗

Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators.Semantic proximity Read ↗

Can correcting the ΔF508-CFTR proteostasis-defect rescue CF lung disease?Semantic proximity Read ↗

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.Semantic proximity Read ↗

Lipid-driven CFTR clustering is impaired in cystic fibrosis and restored by corrector drugs.Semantic proximity Read ↗

Matrine modulates HSC70 levels and rescues ΔF508-CFTR.Semantic proximity Read ↗

Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.