CHIP is associated with Parkin, a gene responsible for familial Parkinson's disease, and enhances its ubiquitin ligase activity

Mol Cell. 2002 Jul;10(1):55-67. doi: 10.1016/s1097-2765(02)00583-x.

Abstract

Unfolded Pael receptor (Pael-R) is a substrate of the E3 ubiquitin ligase Parkin. Accumulation of Pael-R in the endoplasmic reticulum (ER) of dopaminergic neurons induces ER stress leading to neurodegeneration. Here, we show that CHIP, Hsp70, Parkin, and Pael-R formed a complex in vitro and in vivo. The amount of CHIP in the complex was increased during ER stress. CHIP promoted the dissociation of Hsp70 from Parkin and Pael-R, thus facilitating Parkin-mediated Pael-R ubiquitination. Moreover, CHIP enhanced Parkin-mediated in vitro ubiquitination of Pael-R in the absence of Hsp70. Furthermore, CHIP enhanced the ability of Parkin to inhibit cell death induced by Pael-R. Taken together, these results indicate that CHIP is a mammalian E4-like molecule that positively regulates Parkin E3 activity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blotting, Western
  • Cell Death
  • Endoplasmic Reticulum / metabolism
  • Gene Expression Regulation
  • HSP70 Heat-Shock Proteins / metabolism
  • Humans
  • Ligases / genetics
  • Ligases / metabolism*
  • Macromolecular Substances
  • Male
  • Mice
  • Models, Biological
  • Parkinsonian Disorders / enzymology*
  • Parkinsonian Disorders / genetics*
  • Parkinsonian Disorders / pathology
  • Protein Binding
  • Protein Folding
  • Protein Transport
  • Rats
  • Rats, Wistar
  • Substantia Nigra / ultrastructure
  • Transfection
  • Tumor Cells, Cultured
  • Ubiquitin-Protein Ligases

Substances

  • HSP70 Heat-Shock Proteins
  • Macromolecular Substances
  • STUB1 protein, human
  • Ubiquitin-Protein Ligases
  • parkin protein
  • Ligases