ArticleProceedings of the National Academy of Sciences of the United States of America2001
Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
What it found
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Who cites it
28 citing papers in PubMed, 87 citations in OpenAlex.
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- The Golgi as a "Proton Sink" in Cancer.Frontiers in cell and developmental biology · 2021Article
- The Infectious Bronchitis Coronavirus Envelope Protein Alters Golgi pH To Protect the Spike Protein and Promote the Release of Infectious Virus.Journal of virology · 2019Article
- Golgi pH, Ion and Redox Homeostasis: How Much Do They Really Matter?Frontiers in cell and developmental biology · 2019Article
- Article
- Infection, inflammation and host carbohydrates: a Glyco-Evasion Hypothesis.Glycobiology · 2012Review
- Annexin A2 binds to endosomes following organelle destabilization by particulate wear debris.Nature communications · 2012Article
- An advanced stochastic model for mucociliary particle clearance in cystic fibrosis lungs.Journal of thoracic disease · 2012Article
- Glycolipid-Dependent, Protease Sensitive Internalization of Pseudomonas aeruginosa Into Cultured Human Respiratory Epithelial Cells.The open microbiology journal · 2010Article
- Stable, site-specific fluorescent tagging constructs optimized for burkholderia species.Applied and environmental microbiology · 2010Article
- Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages.The Journal of biological chemistry · 2009Article
- Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles.Molecular biology of the cell · 2009Article
- Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis.American journal of physiology. Lung cellular and molecular physiology · 2009Review
- A soluble sulfogalactosyl ceramide mimic promotes Delta F508 CFTR escape from endoplasmic reticulum associated degradation.Chemistry & biology · 2009Article
- Unimpaired lysosomal acidification in respiratory epithelial cells in cystic fibrosis.The Journal of biological chemistry · 2009Article
- Specificity in cancer immunotherapy.Seminars in immunology · 2008Review
- Elevated furin levels in human cystic fibrosis cells result in hypersusceptibility to exotoxin A-induced cytotoxicity.The Journal of clinical investigation · 2007Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
The CFTR gene encodes a transmembrane conductance regulator, which is dysfunctional in patients with cystic fibrosis (CF). The mechanism by which defective CFTR (CF transmembrane conductance regulator) leads to undersialylation of plasma membrane glycoconjugates, which in turn promote lung pathology and colonization with Pseudomonas aeruginosa causing lethal bacterial infections in CF, is not known. Here we show by ratiometric imaging with lumenally exposed pH-sensitive green fluorescent protein that dysfunctional CFTR leads to hyperacidification of the trans-Golgi network (TGN) in CF lung epithelial cells. The hyperacidification of TGN, glycosylation defect of plasma membrane glycoconjugates, and increased P. aeruginosa adherence were corrected by incubating CF respiratory epithelial cells with weak bases. Studies with pharmacological agents indicated a role for sodium conductance, modulated by CFTR regulatory function, in determining the pH of TGN. These studies demonstrate the molecular basis for defective glycosylation of lung epithelial cells and bacterial pathogenesis in CF, and suggest a cure by normalizing the pH of intracellular compartments.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.