"Fibrillin-1" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A fibrillin (FBN1) that functions as a structural support protein for MICROFIBRILS. It also regulates the maturation of OSTEOBLASTS by controlling the availability and concentration of TGF-BETA and BONE MORPHOGENETIC PROTEINS. Mutations in the FBN1 gene are associated with MARFAN SYNDROME.
| Descriptor ID |
D000071838
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| MeSH Number(s) |
D09.400.430.875.500 D12.776.395.341.500 D12.776.860.300.400.500
|
| Concept/Terms |
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Below are MeSH descriptors whose meaning is more general than "Fibrillin-1".
Below are MeSH descriptors whose meaning is more specific than "Fibrillin-1".
This graph shows the total number of publications written about "Fibrillin-1" by people in this website by year, and whether "Fibrillin-1" was a major or minor topic of these publications.
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click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 1999 | 0 | 1 | 1 |
| 2001 | 0 | 2 | 2 |
| 2003 | 0 | 1 | 1 |
| 2004 | 0 | 1 | 1 |
| 2005 | 0 | 1 | 1 |
| 2006 | 0 | 1 | 1 |
| 2007 | 0 | 1 | 1 |
| 2010 | 0 | 2 | 2 |
| 2011 | 0 | 1 | 1 |
| 2012 | 0 | 1 | 1 |
| 2013 | 0 | 1 | 1 |
| 2021 | 0 | 1 | 1 |
| 2022 | 0 | 1 | 1 |
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Below are the most recent publications written about "Fibrillin-1" by people in Profiles.
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Molecular characterization and investigation of the role of genetic variation in phenotypic variability and response to treatment in a large pediatric Marfan syndrome cohort. Genet Med. 2022 05; 24(5):1045-1053.
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Pathophysiology and Pathogenesis of Marfan Syndrome. Adv Exp Med Biol. 2021; 1348:185-206.
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A Pkd1-Fbn1 genetic interaction implicates TGF-? signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2014 Jan; 25(1):81-91.
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Multi-scale biomechanical remodeling in aging and genetic mutant murine mitral valve leaflets: insights into Marfan syndrome. PLoS One. 2012; 7(9):e44639.
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Mitral valve disease in Marfan syndrome and related disorders. J Cardiovasc Transl Res. 2011 Dec; 4(6):741-7.
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Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome. Sci Transl Med. 2010 Mar 17; 2(23):23ra20.
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The pathogenesis of aortopathy in Marfan syndrome and related diseases. Curr Cardiol Rep. 2010 Mar; 12(2):99-107.
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Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome. Circ Res. 2007 Aug 31; 101(5):512-22.
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Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science. 2006 Apr 07; 312(5770):117-21.
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Marfan's syndrome. Lancet. 2005 Dec 03; 366(9501):1965-76.