Transduced HSP27 protein protects neuronal cell death by enhancing FALS-associated SOD1 mutant activity

BMB Rep. 2009 Mar 31;42(3):136-41. doi: 10.5483/bmbrep.2009.42.3.136.

Abstract

Familial Amyotrophic lateral sclerosis (FALS) is a progressive neurodegenetative disorder induced by mutations of the SOD1 gene. Heat shock protein 27 (HSP27) is well-defined as a stress-inducible protein, however the its role in ALS protection has not yet been established. To investigate the role HSP27 may have in SOD1 mutant-mediated apoptosis, human SOD1 or HSP27 genes were fused with a PEP-1 peptide in a bacterial expression vector to produce a genetic in-frame fusion protein, which was then transduced into cells. We found the purified PEP-1-HSP27 fusion proteins can be transduced efficiently into neuronal cells and protect against cell death by enhancing mutant SOD1 activity. Moreover, transduced PEP-1-HSP27 efficiently prevents protein aggregation produced by oxidative stress. These results suggest that transduced HSP27 fusion protein may be explored as a potential therapeutic agent for FALS patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / enzymology*
  • Astrocytes / cytology
  • Astrocytes / metabolism
  • Cell Death
  • Cell Survival
  • Cysteamine / analogs & derivatives
  • Cysteamine / metabolism
  • Cytoprotection*
  • HSP27 Heat-Shock Proteins / metabolism*
  • Heat-Shock Proteins
  • Humans
  • Molecular Chaperones
  • Mutant Proteins / metabolism*
  • Neurons / cytology*
  • Neurons / metabolism
  • Oxidative Stress
  • Peptides / metabolism
  • Protein Structure, Quaternary
  • Recombinant Fusion Proteins / isolation & purification
  • Superoxide Dismutase / chemistry
  • Superoxide Dismutase / metabolism*
  • Superoxide Dismutase-1
  • Transduction, Genetic*

Substances

  • HSP27 Heat-Shock Proteins
  • HSPB1 protein, human
  • Heat-Shock Proteins
  • Molecular Chaperones
  • Mutant Proteins
  • Pep-1 peptide
  • Peptides
  • Recombinant Fusion Proteins
  • SOD1 protein, human
  • Cysteamine
  • Superoxide Dismutase
  • Superoxide Dismutase-1