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Disease relevance of Microfibrils


Psychiatry related information on Microfibrils


High impact information on Microfibrils

  • Microfibrils, either associated with or devoid of elastin, give rise to a variety of extracellular networks in elastic and non-elastic tissues [7].
  • We propose a model for the arrangement of fibrillin monomers in microfibrils that reconciles structural and antibody binding data, and we describe a set of disease-causing mutations that provide the first clues to the specificity of cbEFG interactions [8].
  • Although elastic fibres are known to be composed of microfibril proteins (for example, fibrillins and latent transforming growth factor (TGF)-beta-binding proteins) and polymerized elastin, the mechanism of their assembly and development is not well understood [9].
  • Amyloid P component is located on elastic fibre microfibrils in normal human tissue [10].
  • Calcofluor white ST Alters the in vivo assembly of cellulose microfibrils [2].

Chemical compound and disease context of Microfibrils


Biological context of Microfibrils


Anatomical context of Microfibrils


Associations of Microfibrils with chemical compounds


Gene context of Microfibrils


Analytical, diagnostic and therapeutic context of Microfibrils


  1. Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome. Hollister, D.W., Godfrey, M., Sakai, L.Y., Pyeritz, R.E. N. Engl. J. Med. (1990) [Pubmed]
  2. Calcofluor white ST Alters the in vivo assembly of cellulose microfibrils. Haigler, C.H., Brown, R.M., Benziman, M. Science (1980) [Pubmed]
  3. Toward a molecular understanding of congenital heart disease. Payne, R.M., Johnson, M.C., Grant, J.W., Strauss, A.W. Circulation (1995) [Pubmed]
  4. Abnormalities in fibrillin 1-containing microfibrils in dermal fibroblast cultures from patients with systemic sclerosis (scleroderma). Wallis, D.D., Tan, F.K., Kielty, C.M., Kimball, M.D., Arnett, F.C., Milewicz, D.M. Arthritis Rheum. (2001) [Pubmed]
  5. Kinked collagen VI tetramers and reduced microfibril formation as a result of Bethlem myopathy and introduced triple helical glycine mutations. Lamandé, S.R., Mörgelin, M., Selan, C., Jöbsis, G.J., Baas, F., Bateman, J.F. J. Biol. Chem. (2002) [Pubmed]
  6. Basement membranes, microfibrils and beta amyloid fibrillogenesis in Alzheimer's disease: high resolution ultrastructural findings. Inoue, S., Kuroiwa, M., Kisilevsky, R. Brain Res. Brain Res. Rev. (1999) [Pubmed]
  7. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome. Pereira, L., Andrikopoulos, K., Tian, J., Lee, S.Y., Keene, D.R., Ono, R., Reinhardt, D.P., Sakai, L.Y., Biery, N.J., Bunton, T., Dietz, H.C., Ramirez, F. Nat. Genet. (1997) [Pubmed]
  8. Solution structure of a pair of calcium-binding epidermal growth factor-like domains: implications for the Marfan syndrome and other genetic disorders. Downing, A.K., Knott, V., Werner, J.M., Cardy, C.M., Campbell, I.D., Handford, P.A. Cell (1996) [Pubmed]
  9. Fibulin-5/DANCE is essential for elastogenesis in vivo. Nakamura, T., Lozano, P.R., Ikeda, Y., Iwanaga, Y., Hinek, A., Minamisawa, S., Cheng, C.F., Kobuke, K., Dalton, N., Takada, Y., Tashiro, K., Ross Jr, J., Honjo, T., Chien, K.R. Nature (2002) [Pubmed]
  10. Amyloid P component is located on elastic fibre microfibrils in normal human tissue. Breathnach, S.M., Melrose, S.M., Bhogal, B., de Beer, F.C., Dyck, R.F., Tennent, G., Black, M.M., Pepys, M.B. Nature (1981) [Pubmed]
  11. Biodirected epitaxial nanodeposition of polymers on oriented macromolecular templates. Kondo, T., Nojiri, M., Hishikawa, Y., Togawa, E., Romanovicz, D., Brown, R.M. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  12. A Danish kindred with familial amyloid cardiomyopathy revisited: identification of a mutant transthyretin-methionine111 variant in serum from patients and carriers. Ranløv, I., Alves, I.L., Ranløv, P.J., Husby, G., Costa, P.P., Saraiva, M.J. Am. J. Med. (1992) [Pubmed]
  13. Ultrastructural effects of estrogen replacement on postmenopausal endometrium. Aycock, N.R., Jollie, W.P. Am. J. Obstet. Gynecol. (1979) [Pubmed]
  14. Fibrillary glomerulonephritis. Hsu, B.G., Chang, C.H., Chiang, S.S., Yang, A.H. Zhonghua Yi Xue Za Zhi (Taipei) (2001) [Pubmed]
  15. Mutation or drug-dependent microtubule disruption causes radial swelling without altering parallel cellulose microfibril deposition in Arabidopsis root cells. Sugimoto, K., Himmelspach, R., Williamson, R.E., Wasteneys, G.O. Plant Cell (2003) [Pubmed]
  16. Differential allelic expression of a fibrillin gene (FBN1) in patients with Marfan syndrome. Hewett, D., Lynch, J., Child, A., Firth, H., Sykes, B. Am. J. Hum. Genet. (1994) [Pubmed]
  17. Missense mutations impair intracellular processing of fibrillin and microfibril assembly in Marfan syndrome. Aoyama, T., Tynan, K., Dietz, H.C., Francke, U., Furthmayr, H. Hum. Mol. Genet. (1993) [Pubmed]
  18. Developmental expression of latent transforming growth factor beta binding protein 2 and its requirement early in mouse development. Shipley, J.M., Mecham, R.P., Maus, E., Bonadio, J., Rosenbloom, J., McCarthy, R.T., Baumann, M.L., Frankfater, C., Segade, F., Shapiro, S.D. Mol. Cell. Biol. (2000) [Pubmed]
  19. Mutation of the gene encoding fibrillin-2 results in syndactyly in mice. Chaudhry, S.S., Gazzard, J., Baldock, C., Dixon, J., Rock, M.J., Skinner, G.C., Steel, K.P., Kielty, C.M., Dixon, M.J. Hum. Mol. Genet. (2001) [Pubmed]
  20. Partial sequence of a candidate gene for the Marfan syndrome. Maslen, C.L., Corson, G.M., Maddox, B.K., Glanville, R.W., Sakai, L.Y. Nature (1991) [Pubmed]
  21. Marfan syndrome and mitral valve prolapse. Weyman, A.E., Scherrer-Crosbie, M. J. Clin. Invest. (2004) [Pubmed]
  22. Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. Sakai, L.Y., Keene, D.R., Engvall, E. J. Cell Biol. (1986) [Pubmed]
  23. Versican is expressed in the proliferating zone in the epidermis and in association with the elastic network of the dermis. Zimmermann, D.R., Dours-Zimmermann, M.T., Schubert, M., Bruckner-Tuderman, L. J. Cell Biol. (1994) [Pubmed]
  24. Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. The International Marfan Syndrome Collaborative Study. Tsipouras, P., Del Mastro, R., Sarfarazi, M., Lee, B., Vitale, E., Child, A.H., Godfrey, M., Devereux, R.B., Hewett, D., Steinmann, B. N. Engl. J. Med. (1992) [Pubmed]
  25. Comparison of rhapidosomes and asbestos microfibrils. Hutchison, W.G., Harker, R.I., Allen, M.M. Science (1978) [Pubmed]
  26. Calcofluor white and Congo red inhibit chitin microfibril assembly of Poterioochromonas: evidence for a gap between polymerization and microfibril formation. Herth, W. J. Cell Biol. (1980) [Pubmed]
  27. 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]
  28. COBRA, an Arabidopsis extracellular glycosyl-phosphatidyl inositol-anchored protein, specifically controls highly anisotropic expansion through its involvement in cellulose microfibril orientation. Roudier, F., Fernandez, A.G., Fujita, M., Himmelspach, R., Borner, G.H., Schindelman, G., Song, S., Baskin, T.I., Dupree, P., Wasteneys, G.O., Benfey, P.N. Plant Cell (2005) [Pubmed]
  29. Disruption of the gene encoding the latent transforming growth factor-beta binding protein 4 (LTBP-4) causes abnormal lung development, cardiomyopathy, and colorectal cancer. Sterner-Kock, A., Thorey, I.S., Koli, K., Wempe, F., Otte, J., Bangsow, T., Kuhlmeier, K., Kirchner, T., Jin, S., Keski-Oja, J., von Melchner, H. Genes Dev. (2002) [Pubmed]
  30. Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices. Zhang, H., Apfelroth, S.D., Hu, W., Davis, E.C., Sanguineti, C., Bonadio, J., Mecham, R.P., Ramirez, F. J. Cell Biol. (1994) [Pubmed]
  31. Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils. Zhang, H., Hu, W., Ramirez, F. J. Cell Biol. (1995) [Pubmed]
  32. Regulation of limb patterning by extracellular microfibrils. Arteaga-Solis, E., Gayraud, B., Lee, S.Y., Shum, L., Sakai, L., Ramirez, F. J. Cell Biol. (2001) [Pubmed]
  33. An Arabidopsis endo-1,4-beta-D-glucanase involved in cellulose synthesis undergoes regulated intracellular cycling. Robert, S., Bichet, A., Grandjean, O., Kierzkowski, D., Satiat-Jeunemaître, B., Pelletier, S., Hauser, M.T., Höfte, H., Vernhettes, S. Plant Cell (2005) [Pubmed]
  34. Ultrastructural immunolocalization of lysyl oxidase in vascular connective tissue. Kagan, H.M., Vaccaro, C.A., Bronson, R.E., Tang, S.S., Brody, J.S. J. Cell Biol. (1986) [Pubmed]
  35. 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]
  36. An elastin gene mutation producing abnormal tropoelastin and abnormal elastic fibres in a patient with autosomal dominant cutis laxa. Tassabehji, M., Metcalfe, K., Hurst, J., Ashcroft, G.S., Kielty, C., Wilmot, C., Donnai, D., Read, A.P., Jones, C.J. Hum. Mol. Genet. (1998) [Pubmed]
  37. Co-localization of von Willebrand factor and type VI collagen in human vascular subendothelium. Rand, J.H., Wu, X.X., Potter, B.J., Uson, R.R., Gordon, R.E. Am. J. Pathol. (1993) [Pubmed]
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