Fibrosis: Insights from the Stiff Skin syndrome

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Fibrillin-1 is a 350-kDa glycoprotein component of the extracellular matrix. It is a member of a small family of homologous glycoproteins that includes the fibulins and the latent TGF β-binding proteins (LTBPs). Fibrillin-1 has 43 calciumbinding epidermal growth factor-like (cbEGF-like) domains, 4 noncalcium-binding EGF-like domains, 7 TGF β-binding protein-like (TB) domains, 2 hybrid domains, unique N and C-termini, and a 58-amino-acid proline-rich sequence. Fibrillin-1 is an integral constituent of complex structures in the extracellular matrix called microfibrils [1]. By electron microscopy (EM), microfibrils extracted from tissues or cell culture have a "bead-on-a-string" appearance, with periodically spaced globular domains connected by linear arms [2, 3]. Within the extracellular matrix, microfibrils bind numerous ligands including fibulins, LTBPs, decorin, biglycan, versican, microfibrillar-associated proteins (MAFPs), microfibrilassociated glycoproteins (MAGPs), tropoelastin, heparan sulfate, and some collagens, among others [4]. It is believed that microfibrils form a scaffold for tropoelastin deposition and regulate or inform elastic fiber assembly [5]. Fibrillin-rich microfibrils are found in several elastic and nonelastic tissue types including the skin, vasculature, bones, lungs, and eye.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer US
Pages267-282
Number of pages16
ISBN (Electronic)9781441957740
ISBN (Print)9781441957733
DOIs
StatePublished - Jan 1 2012

Keywords

  • Fibrillin
  • Fibroblast
  • Fibrosis
  • Integrin
  • Skin fibrosis insights
  • Stiff skin syndrome
  • TGF-beta

ASJC Scopus subject areas

  • Medicine(all)

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