The DOI matches its Crossref record; 18 of the 18 references it cites were found in a registry or an index.
Circulating Transforming Growth Factor-β in Marfan Syndrome
Open accessAbstract
Background— Marfan syndrome (MFS) is caused by mutations in the fibrillin-1 gene and dysregulation of transforming growth factor-β (TGF-β). Recent evidence suggests that losartan, an angiotensin II type 1 blocker that blunts TGF-β activation, may be an effective treatment for MFS. We hypothesized that dysregulation of TGF-β might be mirrored in circulating TGF-β concentrations. Methods and Results— Serum obtained from MFS mutant mice ( Fbn1 C1039G /+ ) treated with losartan was analyzed for circulating TGF-β1 concentrations and compared with those from placebo-treated and wild-type mice. Aortic root size was measured by echocardiography. Data were validated in patients with MFS and healthy individuals. In mice, circulating total TGF-β1 concentrations increased with age and were elevated in older untreated Fbn1 C1039G /+ mice compared with wild-type mice ( P =0.01; n=16; mean±SEM, 115±8 ng/mL versus n=17; mean±SEM, 92±4 ng/mL). Losartan-treated Fbn1 C1039G /+ mice had lower total TGF-β1 concentrations compared with age-matched Fbn1 C1039G /+ mice treated with placebo ( P =0.01; n=18; 90±5 ng/mL), and circulating total TGF-β1 levels were indistinguishable from those of age-matched wild-type mice ( P =0.8). Correlation was observed between circulating TGF-β1 levels and aortic root diameters in Fbn1 C1039G /+ and wild-type mice ( P =0.002). In humans, circulating total TGF-β1 concentrations were elevated in patients with MFS compared with control individuals ( P <0.0001; n=53; 15±1.7 ng/mL versus n=74; 2.5±0.4 ng/mL). MFS patients treated with losartan (n=55) or β-blocker (n=80) showed significantly lower total TGF-β1 concentrations compared with untreated MFS patients ( P ≤0.05). Conclusions— Circulating TGF-β1 concentrations are elevated in MFS and decrease after administration of losartan, β-blocker therapy, or both and therefore might serve as a prognostic and therapeutic marker in MFS.
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Lang, R. M., Bierig, M., Devereux, R. B., Flachskampf, F. A., Foster, E., Pellikka, P. A., Picard, M. H., Roman, M. J., Seward, J., Shanewise, J. S., et al. (2005) · Journal of the American Society of Echocardiography · doi:10.1016/j.echo.2005.10.005 · Cited by 11,318
Munger, J. S., Huang, X., Kawakatsu, H., Griffiths, M. J. D., Dalton, S. L., Wu, J., Pittet, J.-F., Kaminski, N., Garat, C., Matthay, M. A., et al. (1999) · Cell · doi:10.1016/s0092-8674(00)80545-0 · Cited by 2,076
Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene
Dietz, H. C., Cutting, C. R., Pyeritz, R. E., Maslen, C. L., Sakai, L. Y., Corson, G. M., Puffenberger, E. G., Hamosh, A., Nanthakumar, E. J., Curristin, S. M., et al. (1991) · Nature · doi:10.1038/352337a0 · Cited by 2,070
Self-citationLosartan, an AT1 Antagonist, Prevents Aortic Aneurysm in a Mouse Model of Marfan Syndrome
Habashi, J. P., Judge, D. P., Holm, T. M., Cohn, R. D., Loeys, B. L., Cooper, T. K., Myers, L., Klein, E. C., Liu, G., Calvi, C., et al. (2006) · Science · doi:10.1126/science.1124287 · Cited by 1,794
Self-citationDysregulation of TGF-β activation contributes to pathogenesis in Marfan syndrome
Neptune, E. R., Frischmeyer, P. A., Arking, D. E., Myers, L., Bunton, T. E., Gayraud, B., Ramirez, F., Sakai, L. Y., & Dietz, H. C. (2003) · Nature Genetics · doi:10.1038/ng1116 · Cited by 1,404
Self-citationJudge, D. P., & Dietz, H. C. (2005) · The Lancet · doi:10.1016/s0140-6736(05)67789-6 · Cited by 1,210
Self-citationAngiotensin II Blockade and Aortic-Root Dilation in Marfan's Syndrome
Brooke, B. S., Habashi, J. P., Judge, D. P., Patel, N., Loeys, B., & Dietz, H. C. (2008) · New England Journal of Medicine · doi:10.1056/nejmoa0706585 · Cited by 843
Self-citationCohn, R. D., van Erp, C., Habashi, J. P., Soleimani, A. A., Klein, E. C., Lisi, M. T., Gamradt, M., ap Rhys, C. M., Holm, T. M., Loeys, B. L., et al. (2007) · Nature Medicine · doi:10.1038/nm1536 · Cited by 669
Self-citationTGF-β–dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome
Ng, C. M., Cheng, A., Myers, L. A., Martinez-Murillo, F., Jie, C., Bedja, D., Gabrielson, K. L., Hausladen, J. M. W., Mecham, R. P., Judge, D. P., et al. (2004) · Journal of Clinical Investigation · doi:10.1172/jci200422715 · Cited by 588
Self-citationJudge, D. P., Biery, N. J., Keene, D. R., Geubtner, J., Myers, L., Huso, D. L., Sakai, L. Y., & Dietz, H. C. (2004) · Journal of Clinical Investigation · doi:10.1172/jci200420641 · Cited by 475
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Cited by
Teekakirikul, P., Eminaga, S., Toka, O., Alcalai, R., Wang, L., Wakimoto, H., Nayor, M., Konno, T., Gorham, J. M., Wolf, C. M., et al. (2010) · Journal of Clinical Investigation · doi:10.1172/jci42028 · Cited by 448
High prevalence of eosinophilic esophagitis in patients with inherited connective tissue disorders
Abonia, J. P., Wen, T., Stucke, E. M., Grotjan, T., Griffith, M. S., Kemme, K. A., Collins, M. H., Putnam, P. E., Franciosi, J. P., von Tiehl, K. F., et al. (2013) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2013.02.030 · Cited by 195
Davis, M. R., & Summers, K. M. (2012) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2012.07.023 · Cited by 103
TGF-β Induces Wnt10b in Osteoclasts From Female Mice to Enhance Coupling to Osteoblasts
Ota, K., Quint, P., Ruan, M., Pederson, L., Westendorf, J. J., Khosla, S., & Oursler, M. J. (2013) · Endocrinology · doi:10.1210/en.2013-1272 · Cited by 86
Transforming growth factor-β and inflammation in vascular (type IV) Ehlers-Danlos syndrome
Morissette, R., Schoenhoff, F., Xu, Z., Shilane, D. A., Griswold, B. F., Chen, W., Yang, J., Zhu, J., Fert-Bober, J., Sloper, L., et al. (2014) · Circulation: Cardiovascular Genetics · doi:10.1161/circgenetics.113.000280 · Cited by 51
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Metadata
- Authors
- Matt, P., Schoenhoff, F., Habashi, J., Holm, T., Van Erp, C., Loch, D., Carlson, O. D., Griswold, B. F., Fu, Q., De Backer, J., Loeys, B., Huso, D. L., McDonnell, N. B., Van Eyk, J. E., Dietz, H. C., & the GenTAC Consortium
- Venue
- Circulation
- Published
- 2009-08-11
- Volume
- 120
- Issue
- 6
- Pages
- 526-532
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2018492695
- Cited by
- 274
- References
- 18
- FWCI
- 10.87
- Type
- journal article
- Language
- en
- Open access
- green
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- Matt, P., Schoenhoff, F., Habashi, J., Holm, T., Van Erp, C., Loch, D., Carlson, O. D., Griswold, B. F., Fu, Q., De Backer, J., Loeys, B., Huso, D. L., McDonnell, N. B., Van Eyk, J. E., Dietz, H. C., & the GenTAC Consortium. (2009). Circulating Transforming Growth Factor-β in Marfan Syndrome. Circulation. https://doi.org/10.1161/circulationaha.108.841981
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