The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Cystic fibrosis: a mucosal immunodeficiency syndrome
Open accessAbstract
Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
The Involvement of Cell-to-Cell Signals in the Development of a Bacterial Biofilm
Davies, D. G., Parsek, M. R., Pearson, J. P., Iglewski, B. H., Costerton, J. W., & Greenberg, E. P. (1998) · Science · doi:10.1126/science.280.5361.295 · Cited by 3,396
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
Saiman, L., Marshall, B. C., Mayer-Hamblett, N., Burns, J. L., Quittner, A. L., Cibene, D. A., Coquillette, S., Fieberg, A. Y., Accurso, F. J., Campbell III, P. W., et al. (2003) · JAMA · doi:10.1001/jama.290.13.1749 · Cited by 958
Luciani, A., Villella, V. R., Esposito, S., Brunetti-Pierri, N., Medina, D., Settembre, C., Gavina, M., Pulze, L., Giardino, I., Pettoello-Mantovani, M., et al. (2010) · Nature Cell Biology · doi:10.1038/ncb2090 · Cited by 467
Analysis of quorum sensing-deficient clinical isolates of Pseudomonas aeruginosa
Schaber, J. A., Carty, N. L., McDonald, N. A., Graham, E. D., Cheluvappa, R., Griswold, J. A., & Hamood, A. N. (2004) · Journal of Medical Microbiology · doi:10.1099/jmm.0.45617-0 · Cited by 224
Zheng, S., De, B. P., Choudhary, S., Comhair, S. A. A., Goggans, T., Slee, R., Williams, B. R. G., Pilewski, J., Haque, S. J., & Erzurum, S. C. (2003) · Immunity · doi:10.1016/s1074-7613(03)00114-6 · Cited by 125
Nuclear Factor-κB p50 Limits Inflammation and Prevents Lung Injury during Escherichia coli Pneumonia
Mizgerd, J. P., Lupa, M. M., Kogan, M. S., Warren, H. B., Kobzik, L., & Topulos, G. P. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200303-412oc · Cited by 66
Cited by
Elborn, J. S. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00576-6 · Cited by 1,805
Pseudomonas aeruginosa : new insights into pathogenesis and host defenses
Gellatly, S. L., & Hancock, R. E. W. (2013) · Pathogens and Disease · doi:10.1111/2049-632x.12033 · Cited by 1,295
Intestinal alkaline phosphatase: novel functions and protective effects
Lallès, J.-P. (2014) · Nutrition Reviews · doi:10.1111/nure.12082 · Cited by 326
Kahl, B. C., Becker, K., & Löffler, B. (2016) · Clinical Microbiology Reviews · doi:10.1128/cmr.00069-15 · Cited by 325
Use of precision cut lung slices as a translational model for the study of lung biology
Liu, G., Betts, C., Cunoosamy, D. M., Åberg, P. M., Hornberg, J. J., Sivars, K. B., & Cohen, T. S. (2019) · Respiratory Research · doi:10.1186/s12931-019-1131-x · Cited by 206
Self-citationMesenchymal stem cells in fibrotic diseases—the two sides of the same coin
Qin, L., Liu, N., Bao, C.-L.-M., Yang, D.-Z., Ma, G.-X., Yi, W.-H., Xiao, G.-Z., & Cao, H.-L. (2023) · Acta Pharmacologica Sinica · doi:10.1038/s41401-022-00952-0 · Cited by 114
Chemically modified hCFTR mRNAs recuperate lung function in a mouse model of cystic fibrosis
Haque, A. K. M. A., Dewerth, A., Antony, J. S., Riethmüller, J., Schweizer, G. R., Weinmann, P., Latifi, N., Yasar, H., Pedemonte, N., Sondo, E., et al. (2018) · Scientific Reports · doi:10.1038/s41598-018-34960-0 · Cited by 80
Reighard, K. P., Hill, D. B., Dixon, G. A., Worley, B. V., & Schoenfisch, M. H. (2015) · Biofouling · doi:10.1080/08927014.2015.1107548 · Cited by 53
Brunel, S. F., Willment, J. A., Brown, G. D., Devereux, G., & Warris, A. (2018) · ERJ Open Research · doi:10.1183/23120541.00068-2017 · Cited by 17
Metadata
- Authors
- Cohen, T. S., & Prince, A.
- Venue
- Nature Medicine
- Published
- 2012-04
- Volume
- 18
- Issue
- 4
- Pages
- 509-519
- ISSN
- 1078-8956 (ISSN-L)
- 1546-170X
- OpenAlex
- W2040233646
- Cited by
- 484
- References
- 8
- FWCI
- 35.57
- Type
- journal article
- Language
- en
- Open access
- green
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
