Congo red, doxycycline, and HSP70 overexpression reduce aggregate formation and cell death in cell models of oculopharyngeal muscular dystrophy

J Med Genet. 2004 Jan;41(1):47-51. doi: 10.1136/jmg.2003.014548.
No abstract available

Publication types

  • Letter
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Animals
  • COS Cells / chemistry
  • COS Cells / metabolism
  • Cell Death / drug effects
  • Cell Death / genetics
  • Cell Line
  • Chlorocebus aethiops
  • Congo Red / chemistry*
  • Doxycycline / pharmacology*
  • Female
  • HSP70 Heat-Shock Proteins / biosynthesis*
  • HSP70 Heat-Shock Proteins / immunology
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies / drug effects
  • Inclusion Bodies / metabolism
  • Male
  • Middle Aged
  • Muscular Dystrophy, Oculopharyngeal / genetics*
  • Muscular Dystrophy, Oculopharyngeal / metabolism
  • Pharyngeal Muscles / chemistry
  • Pharyngeal Muscles / metabolism
  • Poly(A)-Binding Protein II / analysis
  • Poly(A)-Binding Protein II / genetics
  • Poly(A)-Binding Protein II / immunology

Substances

  • HSP70 Heat-Shock Proteins
  • Poly(A)-Binding Protein II
  • Congo Red
  • Doxycycline