Fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 are strongly expressed in the epithelium of human granular and lattice type I corneal dystrophies

Mol Vis. 2012;18:1927-36. Epub 2012 Jul 18.

Abstract

Purpose: To determine the extracellular matrix proteins involved in the formation of human granular and lattice type I corneal stromal dystrophies, the expression patterns of fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 were compared in human corneal stromal dystrophy samples.

Methods: Ten cases of granular dystrophy, 7 cases of lattice dystrophy, and 6 normal corneal buttons collected during corneal transplantation were examined for their expression patterns of fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 by immunohistochemistry.

Results: Highly elevated fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 were observed in the epithelial layer of both granular and lattice type I dystrophies. Fibrillin-2, tenascin-C, and matrilin-4 in the granular dystrophy and all antibodies in the lattice dystrophy showed statistically significant staining in the corneal stroma (p<0.05). Interestingly, fibrillin-2, matrilin-2, and matrilin-4 stained significantly in amyloid plaques of lattice type 1 dystrophy.

Conclusions: Fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 may be markers of the pathogenesis of either granular or lattice type I corneal dystrophy, as revealed by immunohistochemical analysis. Each molecule seems to be involved in the regeneration and reorganization of the corneal matrix in granular and lattice type I dystrophies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Case-Control Studies
  • Corneal Dystrophies, Hereditary / genetics*
  • Corneal Dystrophies, Hereditary / metabolism
  • Corneal Dystrophies, Hereditary / pathology
  • Corneal Stroma / metabolism
  • Corneal Stroma / pathology
  • Epithelium, Corneal / metabolism
  • Epithelium, Corneal / pathology
  • Extracellular Matrix Proteins / genetics*
  • Extracellular Matrix Proteins / metabolism
  • Female
  • Fibrillin-2
  • Fibrillins
  • Gene Expression
  • Genetic Markers
  • Glycoproteins / genetics*
  • Glycoproteins / metabolism
  • Humans
  • Immunohistochemistry
  • Matrilin Proteins
  • Microfilament Proteins / genetics*
  • Microfilament Proteins / metabolism
  • Tenascin / genetics*
  • Tenascin / metabolism
  • Up-Regulation

Substances

  • Extracellular Matrix Proteins
  • FBN2 protein, human
  • Fibrillin-2
  • Fibrillins
  • Genetic Markers
  • Glycoproteins
  • MATN2 protein, human
  • MATN4 protein, human
  • Matrilin Proteins
  • Microfilament Proteins
  • Tenascin