Induction of expression and co-localization of heat shock polypeptides with the polyalanine expansion mutant of poly(A)-binding protein N1 after chemical stress

Biochem Biophys Res Commun. 2008 May 23;370(1):11-5. doi: 10.1016/j.bbrc.2008.02.162. Epub 2008 Mar 14.


Formation of nuclear inclusions consisting of aggregates of a polyalanine expansion mutant of nuclear poly(A)-binding protein (PABPN1) is the hallmark of oculopharyngeal muscular dystrophy (OPMD). OPMD is a late onset autosomal dominant disease. Patients with this disorder exhibit progressive swallowing difficulty and drooping of their eye lids, which starts around the age of 50. Previously we have shown that treatment of cells expressing the mutant PABPN1 with a number of chemicals such as ibuprofen, indomethacin, ZnSO(4), and 8-hydroxy-quinoline induces HSP70 expression and reduces PABPN1 aggregation. In these studies we have shown that expression of additional HSPs including HSP27, HSP40, and HSP105 were induced in mutant PABPN1 expressing cells following exposure to the chemicals mentioned above. Furthermore, all three additional HSPs were translocated to the nucleus and probably helped to properly fold the mutant PABPN1 by co-localizing with this protein.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Nucleus / chemistry
  • Cell Nucleus / metabolism*
  • Green Fluorescent Proteins / analysis
  • Green Fluorescent Proteins / genetics
  • HSP110 Heat-Shock Proteins / analysis
  • HSP110 Heat-Shock Proteins / metabolism
  • HSP27 Heat-Shock Proteins
  • HSP40 Heat-Shock Proteins / analysis
  • HSP40 Heat-Shock Proteins / metabolism
  • HeLa Cells
  • Heat-Shock Proteins / analysis
  • Heat-Shock Proteins / metabolism*
  • Humans
  • Ibuprofen / pharmacology
  • Indomethacin / pharmacology
  • Molecular Chaperones
  • Mutation
  • Neoplasm Proteins / analysis
  • Neoplasm Proteins / metabolism*
  • Oxyquinoline / pharmacology
  • Peptides / analysis
  • Peptides / metabolism
  • Poly(A)-Binding Protein II / analysis
  • Poly(A)-Binding Protein II / genetics
  • Poly(A)-Binding Protein II / metabolism*
  • Transfection
  • Zinc Sulfate / pharmacology


  • DNAJB1 protein, human
  • HSP110 Heat-Shock Proteins
  • HSP27 Heat-Shock Proteins
  • HSP40 Heat-Shock Proteins
  • HSPB1 protein, human
  • HSPH1 protein, human
  • Heat-Shock Proteins
  • Molecular Chaperones
  • Neoplasm Proteins
  • Peptides
  • Poly(A)-Binding Protein II
  • Green Fluorescent Proteins
  • polyalanine
  • Oxyquinoline
  • Zinc Sulfate
  • Ibuprofen
  • Indomethacin