"Fibrillins" 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 family of extracellular matrix glycoproteins that is structurally similar to LATENT TGF-BETA BINDING PROTEINS, but contain additional TGF-beta binding domains, in addition to unique domains at their N and C-terminals. Fibrillins assemble into 10-12 nm MICROFIBRILS that function in a variety of cell interactions with the EXTRACELLULAR MATRIX and developmental processes such as ELASTIC TISSUE maintenance and assembly, and the targeting of growth factors to the extracellular matrix.
| Descriptor ID |
D000071837
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| MeSH Number(s) |
D09.400.430.875 D12.776.395.341 D12.776.860.300.400
|
| Concept/Terms |
|
Below are MeSH descriptors whose meaning is more general than "Fibrillins".
Below are MeSH descriptors whose meaning is more specific than "Fibrillins".
This graph shows the total number of publications written about "Fibrillins" by people in this website by year, and whether "Fibrillins" was a major or minor topic of these publications.
To see the data from this visualization as text,
click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 1999 | 0 | 1 | 1 |
| 2001 | 0 | 1 | 1 |
| 2008 | 0 | 1 | 1 |
| 2009 | 0 | 1 | 1 |
| 2010 | 0 | 1 | 1 |
| 2011 | 0 | 1 | 1 |
| 2012 | 0 | 1 | 1 |
| 2013 | 0 | 2 | 2 |
| 2014 | 0 | 2 | 2 |
| 2015 | 0 | 3 | 3 |
| 2021 | 0 | 1 | 1 |
| 2022 | 0 | 2 | 2 |
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Below are the most recent publications written about "Fibrillins" by people in Profiles.
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A clinical scoring system for early onset (neonatal) Marfan syndrome. Genet Med. 2022 07; 24(7):1503-1511.
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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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Marfan syndrome. Nat Rev Dis Primers. 2021 09 02; 7(1):64.
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Whole exome sequencing confirms the clinical diagnosis of Marfan syndrome combined with X-linked hypophosphatemia. J Transl Med. 2015 Jun 04; 13:179.
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Aortic Disease Presentation and Outcome Associated With ACTA2 Mutations. Circ Cardiovasc Genet. 2015 Jun; 8(3):457-64.
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FBN1 contributing to familial congenital diaphragmatic hernia. Am J Med Genet A. 2015 Apr; 167A(4):831-6.
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Corneal stroma microfibrils. Exp Eye Res. 2015 Mar; 132:198-207.
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Rare and common variants in extracellular matrix gene Fibrillin 2 (FBN2) are associated with macular degeneration. Hum Mol Genet. 2014 Nov 01; 23(21):5827-37.
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Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome. J Clin Invest. 2014 Mar; 124(3):1329-39.
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Thoracic aortic aneurysm frequency and dissection are associated with fibrillin-1 fragment concentrations in circulation. Circ Res. 2013 Oct 25; 113(10):1159-68.