The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
Alveolar Surfactant Homeostasis and the Pathogenesis of Pulmonary Disease
Open accessAbstract
The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of pulmonary function depends on the function of type II epithelial cells that synthesize and secrete pulmonary surfactant lipids and proteins, reducing the collapsing forces created at the air-liquid interface in the alveoli. Genetic and acquired disorders associated with the surfactant system cause both acute and chronic lung disease. Mutations in the ABCA3, SFTPA, SFTPB, SFTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal respiratory failure and chronic interstitial lung disease. Defects in GM-CSF receptor function disrupt surfactant clearance, causing pulmonary alveolar proteinosis. Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases.
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Citations
Cites
Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis
Armanios, M. Y., Chen, J. J.-L., Cogan, J. D., Alder, J. K., Ingersoll, R. G., Markin, C., Lawson, W. E., Xie, M., Vulto, I., Phillips, J. A., et al. (2007) · New England Journal of Medicine · doi:10.1056/nejmoa066157 · Cited by 1,357
One step at a time: endoplasmic reticulum-associated degradation
Vembar, S. S., & Brodsky, J. L. (2008) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2546 · Cited by 1,299
Adult-onset pulmonary fibrosis caused by mutations in telomerase
Tsakiri, K. D., Cronkhite, J. T., Kuan, P. J., Xing, C., Raghu, G., Weissler, J. C., Rosenblatt, R. L., Shay, J. W., & Garcia, C. K. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0701009104 · Cited by 894 · cited 7×
“Heterozygous mutations in genes encoding two essential components of telomerase, TERT (OMIM #187270) and telomerase RNA component (TERC, OMIM #602322), are also associated with pulmonary fibrosis (12, 13).”
InfluentialTelomere Shortening in Familial and Sporadic Pulmonary Fibrosis
Cronkhite, J. T., Xing, C., Raghu, G., Chin, K. M., Torres, F., Rosenblatt, R. L., & Kim Garcia, C. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200804-550oc · Cited by 573
Cited by
Islam, M. N., Das, S. R., Emin, M. T., Wei, M., Sun, L., Westphalen, K., Rowlands, D. J., Quadri, S. K., Bhattacharya, S., & Bhattacharya, J. (2012) · Nature Medicine · doi:10.1038/nm.2736 · Cited by 1,585
Respiratory epithelial cells orchestrate pulmonary innate immunity
Whitsett, J. A., & Alenghat, T. (2015) · Nature Immunology · doi:10.1038/ni.3045 · Cited by 801
Self-citationMacrophages in health and disease
Park, M. D., Silvin, A., Ginhoux, F., & Merad, M. (2022) · Cell · doi:10.1016/j.cell.2022.10.007 · Cited by 644
Efficient generation of lung and airway epithelial cells from human pluripotent stem cells
Huang, S. X. L., Islam, M. N., O'Neill, J., Hu, Z., Yang, Y.-G., Chen, Y.-W., Mumau, M., Green, M. D., Vunjak-Novakovic, G., Bhattacharya, J., et al. (2014) · Nature Biotechnology · doi:10.1038/nbt.2754 · Cited by 567
Lung organoids: current uses and future promise
Barkauskas, C. E., Chung, M.-I., Fioret, B., Gao, X., Katsura, H., & Hogan, B. L. M. (2017) · Development · doi:10.1242/dev.140103 · Cited by 444
Metadata
- Authors
- Whitsett, J. A., Wert, S. E., & Weaver, T. E.
- Venue
- Annual Review of Medicine
- Publisher
- Annual Reviews
- Published
- 2010-02-01
- Volume
- 61
- Issue
- 1
- Pages
- 105-119
- ISSN
- 0066-4219 (ISSN-L)
- 1545-326X
- OpenAlex
- W2142973230
- Cited by
- 487
- References
- 4
- FWCI
- 4.64
- Type
- journal article
- Language
- en
- Open access
- green
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- Whitsett, J. A., Wert, S. E., & Weaver, T. E. (2010). Alveolar Surfactant Homeostasis and the Pathogenesis of Pulmonary Disease. Annual Review of Medicine. https://doi.org/10.1146/annurev.med.60.041807.123500
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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.