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Gene Review

FBN1  -  fibrillin 1

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Disease relevance of FBN1


High impact information on FBN1


Biological context of FBN1


Anatomical context of FBN1

  • In cases of vascular calcification, the decreased expression of TE, FBN1, and LO may be partially responsible for decreased vascular elasticity and also for the decreased formation of new elastic fibers [10].
  • The recent characterization of multiple fibrillin genes raises the question of whether each of the fibrillin proteins is a component of elastic fiber microfibrils and whether their expression during development of elastic tissues is consistent with a function associated with elastogenesis [8].
  • RESULTS: Both MAGP and FBN1 are expressed by the urothelium and are found in association with the underlying basement membrane, as visualized by their co-localization with type IV collagen [11].
  • In contrast, adhesion of auricular chondroblasts to fibrillin-1 was only partially inhibited by these reagents, suggesting that some cell types recognize a second, non-RGD binding site within the fibrillin molecule [12].
  • Fibroblast attachment to fibrillin-1 was sensitive to inhibition by antibodies to the alphavbeta3 receptor and by peptides encoding the RGD sequence in fibrillin-1 and the second RGD sequence in fibrillin-2 [12].

Associations of FBN1 with chemical compounds

  • Poly(A+) ribonucleic acid was isolated from cultured urothelial cells and subjected to Northern analysis using specific complementary deoxyribonucleic acid probes for MAGP and FBN1 [11].
  • MP340 was shown to absorb out completely the microfibrillar immunoreactivity of anti-(reductive guanidine hydrochloride extract) antibodies, indicating that MP340 was (a) the major microfibrillar constituent in these extracts and (b) the second unidentified microfibrillar antigen described previously [9].
  • Cyanogen bromide peptide mapping indicated that MAGP was structurally related to MP340 [9].
  • Role of Ca(2+) for the mechanical properties of fibrillin [13].
  • These experiments indicate that chondroitin sulphate proteoglycans associate with fibrillin and contribute to microfibril assembly [14].

Other interactions of FBN1


Analytical, diagnostic and therapeutic context of FBN1


  1. Identification of a major microfibril-associated glycoprotein-1-binding domain in fibrillin-2. Werneck, C.C., Trask, B.C., Broekelmann, T.J., Trask, T.M., Ritty, T.M., Segade, F., Mecham, R.P. J. Biol. Chem. (2004) [Pubmed]
  2. The molecular genetics of cardiovascular disease. Anderson, P.A. Curr. Opin. Cardiol. (1995) [Pubmed]
  3. The supramolecular organization of fibrillin-rich microfibrils. Baldock, C., Koster, A.J., Ziese, U., Rock, M.J., Sherratt, M.J., Kadler, K.E., Shuttleworth, C.A., Kielty, C.M. J. Cell Biol. (2001) [Pubmed]
  4. Calcium determines the supramolecular organization of fibrillin-rich microfibrils. Wess, T.J., Purslow, P.P., Sherratt, M.J., Ashworth, J., Shuttleworth, C.A., Kielty, C.M. J. Cell Biol. (1998) [Pubmed]
  5. Bovine latent transforming growth factor beta 1-binding protein 2: molecular cloning, identification of tissue isoforms, and immunolocalization to elastin-associated microfibrils. Gibson, M.A., Hatzinikolas, G., Davis, E.C., Baker, E., Sutherland, G.R., Mecham, R.P. Mol. Cell. Biol. (1995) [Pubmed]
  6. Sequence of the coding region of the bovine fibrillin cDNA and localization to bovine chromosome 10. Tilstra, D.J., Li, L., Potter, K.A., Womack, J., Byers, P.H. Genomics (1994) [Pubmed]
  7. Linkage mapping of FBN1 to bovine chromosome 10. Thue, T.D., Buchanan, F.C. Anim. Genet. (2003) [Pubmed]
  8. Fibrillin-1 and fibrillin-2 show temporal and tissue-specific regulation of expression in developing elastic tissues. Mariencheck, M.C., Davis, E.C., Zhang, H., Ramirez, F., Rosenbloom, J., Gibson, M.A., Parks, W.C., Mecham, R.P. Connect. Tissue Res. (1995) [Pubmed]
  9. The protein components of the 12-nanometer microfibrils of elastic and nonelastic tissues. Gibson, M.A., Kumaratilake, J.S., Cleary, E.G. J. Biol. Chem. (1989) [Pubmed]
  10. Accelerated calcification represses the expression of elastic fiber components and lysyl oxidase in cultured bovine aortic smooth muscle cells. Sugitani, H., Wachi, H., Mecham, R.P., Seyama, Y. J. Atheroscler. Thromb. (2002) [Pubmed]
  11. Expression of microfibrillar proteins by bovine bladder urothelium. Rosenbloom, J., Abrams, W.R., Rosenbloom, J., Kucich, U., Decker, S., Mecham, R., Macarak, E., Howard, P. Urology (1997) [Pubmed]
  12. Cell-type specific recognition of RGD- and non-RGD-containing cell binding domains in fibrillin-1. Sakamoto, H., Broekelmann, T., Cheresh, D.A., Ramirez, F., Rosenbloom, J., Mecham, R.P. J. Biol. Chem. (1996) [Pubmed]
  13. Role of Ca(2+) for the mechanical properties of fibrillin. Eriksen, T.A., Wright, D.M., Purslow, P.P., Duance, V.C. Proteins (2001) [Pubmed]
  14. Fibrillin: evidence that chondroitin sulphate proteoglycans are components of microfibrils and associate with newly synthesised monomers. Kielty, C.M., Whittaker, S.P., Shuttleworth, C.A. FEBS Lett. (1996) [Pubmed]
  15. Microfibril-associated glycoprotein-2 (MAGP-2) is specifically associated with fibrillin-containing microfibrils but exhibits more restricted patterns of tissue localization and developmental expression than its structural relative MAGP-1. Gibson, M.A., Finnis, M.L., Kumaratilake, J.S., Cleary, E.G. J. Histochem. Cytochem. (1998) [Pubmed]
  16. The pattern of fibrillin deposition correlates with microfibril-associated glycoprotein 1 (MAGP-1) expression in cultured blood and lymphatic endothelial cells. Weber, E., Rossi, A., Solito, R., Aglianò, M., Sacchi, G., Gerli, R. Lymphology. (2004) [Pubmed]
  17. Marfan syndrome in the third Millennium. Collod-Béroud, G., Boileau, C. Eur. J. Hum. Genet. (2002) [Pubmed]
  18. Evidence for the intramolecular pleating model of fibrillin microfibril organisation from single particle image analysis. Lu, Y., Holmes, D.F., Baldock, C. J. Mol. Biol. (2005) [Pubmed]
  19. Substrate-dependent morphology of supramolecular assemblies: fibrillin and type-VI collagen microfibrils. Sherratt, M.J., Holmes, D.F., Shuttleworth, C.A., Kielty, C.M. Biophys. J. (2004) [Pubmed]
  20. Scanning transmission electron microscopy mass analysis of fibrillin-containing microfibrils from foetal elastic tissues. Sherratt, M.J., Holmes, D.F., Shuttleworth, C.A., Kielty, C.M. Int. J. Biochem. Cell Biol. (1997) [Pubmed]
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