"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
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| Concept/Terms |
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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.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2014 | 0 | 2 | 2 |
| 2015 | 0 | 1 | 1 |
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Below are the most recent publications written about "Fibrillins" by people in Profiles.
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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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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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A novel mutation (C1425Y) in the FBN2 gene in a father and son with congenital contractural arachnodactyly. Genet Test Mol Biomarkers. 2009 Jun; 13(3):295-300.
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Targeting TGF-beta and the extracellular matrix in Marfan's syndrome. Dev Cell. 2008 Jul; 15(1):1-2.
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Targeted disruption of NeuroD, a proneural basic helix-loop-helix factor, impairs distal lung formation and neuroendocrine morphology in the neonatal lung. J Biol Chem. 2008 Jul 25; 283(30):21160-9.
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A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation. Genome Res. 1996 Apr; 6(4):300-13.
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Severe neonatal Marfan syndrome resulting from a de novo 3-bp insertion into the fibrillin gene on chromosome 15. Am J Hum Genet. 1994 Mar; 54(3):447-53.