The E3 ligase Smurf1 regulates Wolfram syndrome protein stability at the endoplasmic reticulum.

@article{Guo2011TheEL,
  title={The E3 ligase Smurf1 regulates Wolfram syndrome protein stability at the endoplasmic reticulum.},
  author={Xing Guo and Shan Shen and Shanshan Song and Shan He and Y B Cui and Guichun Xing and Jian Wang and Yuxin Yin and Libin Fan and Fuchu He and Lingqiang Zhang},
  journal={The Journal of biological chemistry},
  year={2011},
  volume={286 20},
  pages={18037-47}
}
The HECT-type ubiquitin ligase (E3) Smad ubiquitination regulatory factor 1 (Smurf1) targets various substrates, including Smad1/5, RhoA, Prickle 1, MEKK2, and JunB for degradation and thereby regulates adult bone formation and embryonic development. Here, we identify the endoplasmic reticulum (ER)-localized Wolfram syndrome protein (WFS1) as a specific degradation substrate of Smurf1. Mutations in the WFS1 gene cause Wolfram syndrome, an autosomal recessive disorder characterized by diabetes… CONTINUE READING
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