ReviewAmerican journal of physiology. Lung cellular and molecular physiology2009
Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis.
Review in American journal of physiology. Lung cellular and molecular physiology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
23 citing papers in PubMed, 57 citations in OpenAlex.
- Altered Degranulation and pH of Neutrophil Phagosomes Impacts Antimicrobial Efficiency in Cystic Fibrosis.Frontiers in immunology · 2020Trial
- Considerations for early life genetic therapies in cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2026Review
- Mechanisms by which the cystic fibrosis transmembrane conductance regulator may influence SARS-CoV-2 infection and COVID-19 disease severity.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2023Article
- Macrophage Lysosomal Alkalinization Drives Invasive Aspergillosis in a Mouse Cystic Fibrosis Model of Airway Transplantation.Journal of fungi (Basel, Switzerland) · 2022Article
- Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2022Article
- Role of Lysosomal Acidification Dysfunction in Mesenchymal Stem Cell Senescence.Frontiers in cell and developmental biology · 2022Review
- Review
- Characterizing chloride-dependent acidification in brain clathrin-coated vesiclesBiochemistry and cell biology = Biochimie et biologie cellulaire · 2019Article
- New ISE-Based Apparatus for NaSensors (Basel, Switzerland) · 2019Article
- Mapping targetable inflammation and outcomes with cystic fibrosis biomarkers.Pediatric pulmonology · 2017Review
- The voltage-gated sodium channel TPC1 confers endolysosomal excitability.Nature chemical biology · 2014Article
- Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β1-integrin signaling and delayed wound repair.American journal of physiology. Cell physiology · 2014Article
- Low levels of insulin-like growth factor-1 contribute to alveolar macrophage dysfunction in cystic fibrosis.Journal of immunology (Baltimore, Md. : 1950) · 2013Article
- CFTR, mucins, and mucus obstruction in cystic fibrosis.Cold Spring Harbor perspectives in medicine · 2012Review
- Functional Rescue of F508del-CFTR Using Small Molecule Correctors.Frontiers in pharmacology · 2012Article
- Abnormal trafficking and degradation of TLR4 underlie the elevated inflammatory response in cystic fibrosis.Journal of immunology (Baltimore, Md. : 1950) · 2011Article
- Listeria monocytogenes exploits cystic fibrosis transmembrane conductance regulator (CFTR) to escape the phagosome.Proceedings of the National Academy of Sciences of the United States of America · 2011Article
- Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa.PloS one · 2011Article
- Ceramide in cystic fibrosis: a potential new target for therapeutic intervention.Journal of lipids · 2011Article
- Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue DeltaF508 cystic fibrosis mouse.American journal of physiology. Gastrointestinal and liver physiology · 2010Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
The cellular mechanisms by which loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel produce cystic fibrosis (CF) lung disease remain uncertain. Defective organellar function has been proposed as an important determinant in the pathogenesis of CF lung disease. According to one hypothesis, reduced CFTR chloride conductance in organelles in CF impairs their acidification by preventing chloride entry into the organelle lumen, which is needed to balance the positive charge produced by proton entry. According to a different hypothesis, CFTR mutation hyperacidifies organelles by an indirect mechanism involving unregulated sodium efflux through epithelial sodium channels. There are reports of defective Golgi, endosomal and lysosomal acidification in CF epithelial cells, defective phagolysosomal acidification in CF alveolar macrophages, and organellar hyperacidification in CF respiratory epithelial cells. The common theme relating too high or low organellar pH to cellular dysfunction and CF pathogenesis is impaired functioning of organellar enzymes, such as those involved in ceramide metabolism and protein processing in epithelial cells and antimicrobial activity in alveolar macrophages. We review here the evidence for defective organellar acidification in CF. Significant technical and conceptual concerns are discussed regarding the validity of data showing too high/low organellar pH in CF cells, and rigorous measurements of organellar pH in CF cells are reviewed that fail to support defective organellar acidification in CF. Indeed, there is an expanding body of evidence supporting the involvement of non-CFTR chloride channels in organellar acidification. We conclude that biologically significant involvement of CFTR in organellar acidification is unlikely.
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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.