Increased gene expression of matrix metalloproteinase-3 (stromelysin) in skin fibroblasts from patients with severe recessive dystrophic epidermolysis bullosa

Biochem Biophys Res Commun. 1991 Jan 31;174(2):1003-8. doi: 10.1016/0006-291x(91)91518-h.

Abstract

Gene expression of matrix metalloproteinase 3 (MMP-3 = stromelysin) was examined in the skin fibroblasts obtained from patients with severe recessive dystrophic epidermolysis bullosa (RDEB). Steady-state mRNA level of MMP-3 was selectively increased in the unstimulated RDEB cells by a post-transcriptional mechanism. A parallel study on the susceptibility of type VII collagen to MMPs revealed that this type of collagen is degraded by MMP-3, but not by MMP-1 (collagenase). These data suggest that MMP-3 may play an important role in the blister formation fo the skin in RDEB patients by the degradation of anchoring fibrils consisting of type VII collagen.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blotting, Northern
  • Cell Nucleus / metabolism
  • Cells, Cultured
  • Epidermolysis Bullosa Dystrophica / enzymology
  • Epidermolysis Bullosa Dystrophica / genetics*
  • Fibroblasts / enzymology
  • Gene Expression
  • Genes, Recessive*
  • Humans
  • Kinetics
  • Matrix Metalloproteinase 3
  • Metalloendopeptidases / genetics*
  • Nucleic Acid Hybridization
  • RNA / genetics
  • RNA / isolation & purification
  • Skin / enzymology*
  • Transcription, Genetic

Substances

  • RNA
  • Metalloendopeptidases
  • Matrix Metalloproteinase 3