"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
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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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Year | Major Topic | Minor Topic | Total |
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2003 | 0 | 1 | 1 |
2005 | 0 | 1 | 1 |
2011 | 0 | 1 | 1 |
2012 | 0 | 1 | 1 |
2015 | 0 | 1 | 1 |
2017 | 1 | 0 | 1 |
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Below are the most recent publications written about "Fibrillin-1" by people in Profiles.
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Newell K, Smith W, Ghoshhajra B, Isselbacher E, Lin A, Lindsay ME. Cervical artery dissection expands the cardiovascular phenotype in FBN1-related Weill-Marchesani syndrome. Am J Med Genet A. 2017 Sep; 173(9):2551-2556.
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Zhang L, Lai YH, Capasso JE, Han S, Levin AV. Early onset ectopia lentis due to a FBN1 mutation with non-penetrance. Am J Med Genet A. 2015 Jun; 167(6):1365-8.
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Teekakirikul P, Milewicz DM, Miller DT, Lacro RV, Regalado ES, Rosales AM, Ryan DP, Toler TL, Lin AE. Thoracic aortic disease in two patients with juvenile polyposis syndrome and SMAD4 mutations. Am J Med Genet A. 2013 Jan; 161A(1):185-91.
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LeMaire SA, McDonald ML, Guo DC, Russell L, Miller CC, Johnson RJ, Bekheirnia MR, Franco LM, Nguyen M, Pyeritz RE, Bavaria JE, Devereux R, Maslen C, Holmes KW, Eagle K, Body SC, Seidman C, Seidman JG, Isselbacher EM, Bray M, Coselli JS, Estrera AL, Safi HJ, Belmont JW, Leal SM, Milewicz DM. Genome-wide association study identifies a susceptibility locus for thoracic aortic aneurysms and aortic dissections spanning FBN1 at 15q21.1. Nat Genet. 2011 Sep 11; 43(10):996-1000.
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Hartner A, Schaefer L, Porst M, Cordasic N, Gabriel A, Klanke B, Reinhardt DP, Hilgers KF. Role of fibrillin-1 in hypertensive and diabetic glomerular disease. Am J Physiol Renal Physiol. 2006 Jun; 290(6):F1329-36.
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Fedak PW, de Sa MP, Verma S, Nili N, Kazemian P, Butany J, Strauss BH, Weisel RD, David TE. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Cardiovasc Surg. 2003 Sep; 126(3):797-806.