Mechanism of BAG1 repair on Parkinson's disease-linked DJ1 mutation

J Biomol Struct Dyn. 2012;30(1):1-12. doi: 10.1080/07391102.2012.674182.

Abstract

Mutant oncogene DJ1 L166P has been linked to a familial form of early-onset Parkinson's disease (PD). The DJ1 mutant deformed C-terminal helices and prevented the formation of a functional DJ1 dimer. Intriguingly, chaperon modulator, BCL2-associated athanogene (BAG1), has been shown to repair DJ1 mutant and restore its functions. Molecular simulation techniques were employed to elucidate protein-protein interactions between BAG1 and DJ1. Interaction of BAG1 with DJ1 showed recovery of disrupted alpha helix structures and H-bonds stabilizing the functional site Cys106. The His126-Pro184 H-bond (hydrogen-bond) critical to maintaining dimer interfaces was also restored and led to the restoration of dimer formation. High conformational to functional DJ1 dimer was confirmed root mean square deviation = 0.74 Å). Results of this suggest several molecular insights on BAG1-DJ1 repair mechanism and may have an impact on advancing PD treatments.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Binding Sites
  • Cystine / chemistry
  • DNA-Binding Proteins / chemistry*
  • DNA-Binding Proteins / metabolism
  • Humans
  • Hydrogen Bonding
  • Intracellular Signaling Peptides and Proteins / chemistry*
  • Intracellular Signaling Peptides and Proteins / genetics*
  • Intracellular Signaling Peptides and Proteins / metabolism
  • Molecular Dynamics Simulation
  • Mutation*
  • Oncogene Proteins / chemistry*
  • Oncogene Proteins / genetics*
  • Oncogene Proteins / metabolism
  • Parkinson Disease / genetics*
  • Parkinson Disease / metabolism
  • Protein Binding
  • Protein Deglycase DJ-1
  • Protein Interaction Domains and Motifs
  • Protein Multimerization
  • Protein Structure, Secondary
  • Reproducibility of Results
  • Transcription Factors / chemistry*
  • Transcription Factors / metabolism

Substances

  • BCL2-associated athanogene 1 protein
  • DNA-Binding Proteins
  • Intracellular Signaling Peptides and Proteins
  • Oncogene Proteins
  • Transcription Factors
  • Cystine
  • PARK7 protein, human
  • Protein Deglycase DJ-1