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

Y Imai, M Soda, S Hatakeyama, T Akagi, T Hashikawa… - Molecular cell, 2002 - cell.com
Y Imai, M Soda, S Hatakeyama, T Akagi, T Hashikawa, KI Nakayama, R Takahashi
Molecular cell, 2002cell.com
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 …
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.
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