fibrillins

Summary

Summary: 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.

Top Publications

  1. De Backer J. The expanding cardiovascular phenotype of Marfan syndrome. Eur J Echocardiogr. 2009;10:213-5 pubmed publisher
  2. Raghunath M, Unsöld C, Kubitscheck U, Bruckner Tuderman L, Peters R, Meuli M. The cutaneous microfibrillar apparatus contains latent transforming growth factor-beta binding protein-1 (LTBP-1) and is a repository for latent TGF-beta1. J Invest Dermatol. 1998;111:559-64 pubmed
    ..Thus, this major connective tissue structure does not only serve as a force bearing element and scaffold for elastin deposition in the dermis, but also as an important repository for latent TGF-beta in the skin. ..
  3. Segars J, DeCherney A. Is there a genetic basis for polycystic ovary syndrome?. J Clin Endocrinol Metab. 2010;95:2058-60 pubmed publisher
  4. Barnett C, Wilson G, Chiasson D, Gross G, Hinek A, Hawkins C, et al. Central nervous system abnormalities in two cases with neonatal Marfan syndrome with novel mutations in the fibrillin-1 gene. Am J Med Genet A. 2010;152A:2409-12 pubmed publisher
  5. Villano M, Borghini A, Manetti M, Gabbrielli E, Rossi A, Sestini P, et al. Systemic sclerosis sera affect fibrillin-1 deposition by dermal blood microvascular endothelial cells: therapeutic implications of cyclophosphamide. Arthritis Res Ther. 2013;15:R90 pubmed publisher
    ..In SSc, CYC treatment might limit fibrosis through the maintenance of physiologic fibrillin-1 synthesis and deposition by B-MVECs. ..
  6. Garg A, Xing C. De novo heterozygous FBN1 mutations in the extreme C-terminal region cause progeroid fibrillinopathy. Am J Med Genet A. 2014;164A:1341-5 pubmed publisher
  7. Kielty C. Fell-Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?. Int J Exp Pathol. 2017;98:172-190 pubmed publisher
    ..This review is a personal reflection on key insights, and puts forward the conceptual hypothesis that microfibrils are structural 'tensometers' that direct cells to monitor and respond to altered tissue mechanics. ..