Structural and functional failure of fibrillin‑1 in human diseases (Review)

  • Authors:
    • Sandra Schrenk
    • Carola Cenzi
    • Thomas Bertalot
    • Maria Teresa Conconi
    • Rosa Di Liddo
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  • Published online on: December 22, 2017     https://doi.org/10.3892/ijmm.2017.3343
  • Pages: 1213-1223
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Abstract

Fibrillins (FBNs) are key relay molecules that form the backbone of microfibrils in elastic and non‑elastic tissues. Interacting with other components of the extracellular matrix (ECM), these ubiquitous glycoproteins exert pivotal roles in tissue development, homeostasis and repair. In addition to mechanical support, FBN networks also exhibit regulatory activities on growth factor signalling, ECM formation, cell behaviour and the immune response. Consequently, mutations affecting the structure, assembly and stability of FBN microfibrils have been associated with impaired biomechanical tissue properties, altered cell‑matrix interactions, uncontrolled growth factor or cytokine activation, and the development of fibrillinopathies and associated severe complications in multiple organs. Beyond a panoramic overview of structural cues of the FBN network, the present review will also describe the pathological implications of FBN disorders in the development of inflammatory and fibrotic conditions.
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March 2018
Volume 41 Issue 3

Print ISSN: 1107-3756
Online ISSN:1791-244X

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APA
Schrenk, S., Cenzi, C., Bertalot, T., Conconi, M.T., & Di Liddo, R. (2018). Structural and functional failure of fibrillin‑1 in human diseases (Review). International Journal of Molecular Medicine, 41, 1213-1223. https://doi.org/10.3892/ijmm.2017.3343
MLA
Schrenk, S., Cenzi, C., Bertalot, T., Conconi, M. T., Di Liddo, R."Structural and functional failure of fibrillin‑1 in human diseases (Review)". International Journal of Molecular Medicine 41.3 (2018): 1213-1223.
Chicago
Schrenk, S., Cenzi, C., Bertalot, T., Conconi, M. T., Di Liddo, R."Structural and functional failure of fibrillin‑1 in human diseases (Review)". International Journal of Molecular Medicine 41, no. 3 (2018): 1213-1223. https://doi.org/10.3892/ijmm.2017.3343