The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Elastic fibres in health and disease
Open accessAbstract
Elastic fibres are insoluble components of the extracellular matrix of dynamic connective tissues such as skin, arteries, lungs and ligaments. They are laid down during development, and comprise a cross-linked elastin core within a template of fibrillin-based microfibrils. Their function is to endow tissues with the property of elastic recoil, and they also regulate the bioavailability of transforming growth factor β. Severe heritable elastic fibre diseases are caused by mutations in elastic fibre components; for example, mutations in elastin cause supravalvular aortic stenosis and autosomal dominant cutis laxa, mutations in fibrillin-1 cause Marfan syndrome and Weill–Marchesani syndrome, and mutations in fibulins-4 and -5 cause autosomal recessive cutis laxa. Acquired elastic fibre defects include dermal elastosis, whereas inflammatory damage to fibres contributes to pathologies such as pulmonary emphysema and vascular disease. This review outlines the latest understanding of the composition and assembly of elastic fibres, and describes elastic fibre diseases and current therapeutic approaches.
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Cites
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Klein, R. J., Zeiss, C., Chew, E. Y., Tsai, J.-Y., Sackler, R. S., Haynes, C., Henning, A. K., SanGiovanni, J. P., Mane, S. M., Mayne, S. T., et al. (2005) · Science · doi:10.1126/science.1109557 · Cited by 4,492
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Tryptophan metabolism in the central nervous system: medical implications
Ruddick, J. P., Evans, A. K., Nutt, D. J., Lightman, S. L., Rook, G. A. W., & Lowry, C. A. (2006) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399406000068 · Cited by 519
The Evolution of Extracellular Matrix
Özbek, S., Balasubramanian, P. G., Chiquet-Ehrismann, R., Tucker, R. P., & Adams, J. C. (2010) · Molecular Biology of the Cell · doi:10.1091/mbc.e10-03-0251 · Cited by 347
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Molecular Basis of Elastic Fiber Formation
Rock, M. J., Cain, S. A., Freeman, L. J., Morgan, A., Mellody, K., Marson, A., Shuttleworth, C. A., Weiss, A. S., & Kielty, C. M. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m400212200 · Cited by 167
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Cited by
Mechanotransduction and extracellular matrix homeostasis
Humphrey, J. D., Dufresne, E. R., & Schwartz, M. A. (2014) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3896 · Cited by 2,124
The extracellular matrix and transforming growth factor-β1: Tale of a strained relationship
Hinz, B. (2015) · Matrix Biology · doi:10.1016/j.matbio.2015.05.006 · Cited by 608
Halper, J., & Kjaer, M. (2014) · Advances in Experimental Medicine and Biology · doi:10.1007/978-94-007-7893-1_3 · Cited by 532
The good and the bad collagens of fibrosis – Their role in signaling and organ function
Karsdal, M. A., Nielsen, S. H., Leeming, D. J., Langholm, L. L., Nielsen, M. J., Manon-Jensen, T., Siebuhr, A., Gudmann, N. S., Rønnow, S., Sand, J. M., et al. (2017) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2017.07.014 · Cited by 509
Hinz, B. (2016) · Experimental Eye Research · doi:10.1016/j.exer.2015.07.009 · Cited by 424
Metadata
- Authors
- Baldwin, A. K., Simpson, A., Steer, R., Cain, S. A., & Kielty, C. M.
- Venue
- Expert Reviews in Molecular Medicine
- Publisher
- Cambridge University Press (CUP)
- Published
- 2013
- Volume
- 15
- Pages
- e8-e8
- ISSN
- 1462-3994 (ISSN-L)
- OpenAlex
- W2145335014
- Cited by
- 427
- References
- 8
- FWCI
- 22.46
- Type
- journal article
- Language
- en
- Open access
- bronze
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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.
