Modulation of polyglutamine inclusion formation by the Hsp70 chaperone machine

Exp Cell Res. 2007 Oct 1;313(16):3568-78. doi: 10.1016/j.yexcr.2007.07.034. Epub 2007 Aug 8.

Abstract

Components of the Hsp70 chaperone machine have been implied in protection against polyglutamine (poly-Q) pathologies. Yet, little is known about specific mechanisms and the rate-limiting components that account for this protective effect. Here, we examined the effects of an Hsp70 chaperone family member (HspA1A) and its cofactors Hsp40 (DnaJB1), Bag-1 and CHIP on poly-Q protein inclusion formation and SDS-insolubilization. Overexpression of HspA1A alone did not suppress inclusion formation, while overexpression of DnaJB1 reduced poly-Q inclusion formation and insolubilization. The reducing effect of DnaJB1 on inclusion formation was enhanced by coexpressing HspA1A, and was dependent on the interaction of DnaJB1 with Hsp70/Hsc70 chaperones. Additionally, two factors connecting Hsp70 activity with protein degradation by the ubiquitin-proteasome system Bag-1 and CHIP slightly decreased the levels of soluble poly-Q protein, but the amount of aggregated protein and fraction of cells with inclusions remained unaltered. Our data suggest that the HspA1A chaperone machine can modulate poly-Q inclusion formation depending on the ratio of its components and that DnaJB1 is the rate-limiting step.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Death
  • Cell Line, Tumor
  • Cell Nucleus / metabolism
  • Cricetinae
  • Green Fluorescent Proteins / metabolism
  • HSP70 Heat-Shock Proteins / metabolism*
  • Humans
  • Inclusion Bodies / metabolism*
  • Mice
  • Nerve Tissue Proteins / metabolism
  • Neuroblastoma / pathology
  • Peptide Fragments / metabolism
  • Peptides / metabolism*
  • Protein Structure, Quaternary
  • Solubility
  • Time Factors

Substances

  • HSP70 Heat-Shock Proteins
  • HSPA1A protein, human
  • Nerve Tissue Proteins
  • Peptide Fragments
  • Peptides
  • enhanced green fluorescent protein
  • Green Fluorescent Proteins
  • polyglutamine