Altered fibronectin mRNA splicing in skin fibroblasts from Ehlers-Danlos syndrome patients: in situ hybridization analysis

Cell Biol Int Rep. 1991 Dec;15(12):1195-206. doi: 10.1016/0309-1651(91)90091-v.

Abstract

The expression of fibronectin (FN) mRNA isoforms generated by alternative splicing of the EDA region was studied by dot-blot and in situ hybridization, using specific FN cDNA probes, in skin fibroblasts from controls and Ehlers-Danlos Syndrome (EDS) patients (types III, IV, VII and non classified). An Image Analysis program was used for the quantitative evaluation and comparison of FN mRNAs levels in the different cell strains. The in situ hybridization analysis showed that FN mRNAs are homogeneously expressed in all cells of each fibroblast strain analyzed. While in control fibroblasts about 70% of FN mRNA isoforms contain the EDA region (EDA+ FN mRNAs), in EDS fibroblasts this fraction is reduced up to about 30%. This indicates that in the EDS fibroblasts analyzed a deregulation of the alternative splicing processes acting at the EDA region takes place.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoradiography
  • Cells, Cultured
  • DNA / genetics
  • DNA Probes
  • Ehlers-Danlos Syndrome / genetics*
  • Ehlers-Danlos Syndrome / metabolism
  • Ehlers-Danlos Syndrome / pathology
  • Fibroblasts / metabolism
  • Fibroblasts / pathology*
  • Fibronectins / genetics*
  • Humans
  • Image Processing, Computer-Assisted
  • Male
  • Nucleic Acid Hybridization
  • RNA Splicing / genetics*
  • RNA, Messenger / genetics*
  • Skin / metabolism
  • Skin / pathology*

Substances

  • DNA Probes
  • Fibronectins
  • RNA, Messenger
  • DNA