The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
Diseases of Pulmonary Surfactant Homeostasis
Open accessAbstract
Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory distress syndrome. Identification of the surfactant proteins, lipid transporters, and transcriptional networks regulating their expression has provided the tools and insights needed to discern the molecular and cellular processes regulating the production and function of pulmonary surfactant prior to and after birth. Mutations in genes regulating surfactant homeostasis have been associated with severe lung disease in neonates and older infants. Biophysical and transgenic mouse models have provided insight into the mechanisms underlying surfactant protein and alveolar homeostasis. These studies have provided the framework for understanding the structure and function of pulmonary surfactant, which has informed understanding of the pathogenesis of diverse pulmonary disorders previously considered idiopathic. This review considers the pulmonary surfactant system and the genetic causes of acute and chronic lung disease caused by disruption of alveolar homeostasis.
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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
A Mutation in the Surfactant Protein C Gene Associated with Familial Interstitial Lung Disease
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Self-citationAdult-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
Short telomeres are a risk factor for idiopathic pulmonary fibrosis
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Telomere Lengths, Pulmonary Fibrosis and Telomerase (TERT) Mutations
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Telomerase and idiopathic pulmonary fibrosis
Armanios, M. (2012) · Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis · doi:10.1016/j.mrfmmm.2011.10.013 · Cited by 245
Parry, E. M., Alder, J. K., Qi, X., Chen, J. J.-L., & Armanios, M. (2011) · Blood · doi:10.1182/blood-2010-11-322149 · Cited by 194
The transcription factor PU.1 is involved in macrophage proliferation.
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Cited by
Thébaud, B., Goss, K. N., Laughon, M., Whitsett, J. A., Abman, S. H., Steinhorn, R. H., Aschner, J. L., Davis, P. G., McGrath-Morrow, S. A., Soll, R. F., et al. (2019) · Nature Reviews Disease Primers · doi:10.1038/s41572-019-0127-7 · Cited by 1,070
Self-citationAn atlas of the aging lung mapped by single cell transcriptomics and deep tissue proteomics
Angelidis, I., Simon, L. M., Fernandez, I. E., Strunz, M., Mayr, C. H., Greiffo, F. R., Tsitsiridis, G., Ansari, M., Graf, E., Strom, T.-M., et al. (2019) · Nature Communications · doi:10.1038/s41467-019-08831-9 · Cited by 637
A three-dimensional model of human lung development and disease from pluripotent stem cells
Chen, Y.-W., Huang, S. X., de Carvalho, A. L. R. T., Ho, S.-H., Islam, M. N., Volpi, S., Notarangelo, L. D., Ciancanelli, M., Casanova, J.-L., Bhattacharya, J., et al. (2017) · Nature Cell Biology · doi:10.1038/ncb3510 · Cited by 608
Xu, Y., Mizuno, T., Sridharan, A., Du, Y., Guo, M., Tang, J., Wikenheiser-Brokamp, K. A., Perl, A.-K. T., Funari, V. A., Gokey, J. J., et al. (2016) · JCI Insight · doi:10.1172/jci.insight.90558 · Cited by 580
Self-citationDifferentiation of Human Pluripotent Stem Cells into Functional Lung Alveolar Epithelial Cells
Jacob, A., Morley, M., Hawkins, F., McCauley, K. B., Jean, J. C., Heins, H., Na, C.-L., Weaver, T. E., Vedaie, M., Hurley, K., et al. (2017) · Cell Stem Cell · doi:10.1016/j.stem.2017.08.014 · Cited by 565
Self-citation
Metadata
- Authors
- Whitsett, J. A., Wert, S. E., & Weaver, T. E.
- Venue
- Annual Review of Pathology: Mechanisms of Disease
- Publisher
- Annual Reviews
- Published
- 2015-01-24
- Volume
- 10
- Issue
- 1
- Pages
- 371-393
- ISSN
- 1553-4006 (ISSN-L)
- 1553-4014
- OpenAlex
- W2071009656
- Cited by
- 256
- References
- 9
- FWCI
- 4.99
- Type
- journal article
- Language
- en
- Open access
- green
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