Post-transcriptional modification of the gene genetically linked to juvenile open-angle glaucoma; novel transcripts in human ocular tissues

Gene. 2001 Dec 12;280(1-2):115-22. doi: 10.1016/s0378-1119(01)00777-6.

Abstract

Mutation in the myocilin gene is associated with 4% (familial form) of glaucoma cases. The underlying mechanism in non-familial cases remains unclear. Myocilin is shown here to undergo a post-transcriptional modification event, giving rise to deletion forms. Data presented show the expression profile of the shorter transcripts occurs in a tissue-specific and donor-specific manner, and the possibility is raised that their expression is disease-associated. Furthermore, demonstration of their upregulation by dexamethasone provide support for a possible role in steroid-induced glaucoma, a model for the disease process of glaucoma.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Base Sequence
  • Blotting, Northern
  • Cell Line
  • Child
  • Child, Preschool
  • Ciliary Body / metabolism
  • Cytoskeletal Proteins
  • DNA, Complementary / chemistry
  • DNA, Complementary / genetics
  • Dexamethasone / pharmacology
  • Eye Proteins / genetics*
  • Female
  • Gene Deletion
  • Gene Expression
  • Gene Expression Regulation / drug effects
  • Glaucoma, Open-Angle / genetics*
  • Glaucoma, Open-Angle / pathology
  • Glycoproteins / genetics*
  • Humans
  • Infant
  • Male
  • Middle Aged
  • RNA Processing, Post-Transcriptional*
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Retina / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA
  • Sequence Deletion
  • Tissue Distribution
  • Trabecular Meshwork / cytology
  • Trabecular Meshwork / drug effects
  • Trabecular Meshwork / metabolism
  • Transcription, Genetic
  • Up-Regulation / drug effects

Substances

  • Cytoskeletal Proteins
  • DNA, Complementary
  • Eye Proteins
  • Glycoproteins
  • RNA, Messenger
  • trabecular meshwork-induced glucocorticoid response protein
  • Dexamethasone