[HTML][HTML] The E3 ligase Smurf1 regulates Wolfram syndrome protein stability at the endoplasmic reticulum

X Guo, S Shen, S Song, S He, Y Cui, G Xing… - Journal of Biological …, 2011 - ASBMB
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 mellitus and optic atrophy. WFS1 …