β-Cell Dysfunction Due to Increased ER Stress in a Stem Cell Model of Wolfram Syndrome

@article{Shang2014CellDD,
  title={$\beta$-Cell Dysfunction Due to Increased ER Stress in a Stem Cell Model of Wolfram Syndrome},
  author={Linshan Shang and Haiqing Hua and Kylie S Foo and Hector Martinez and Kazuhisa Watanabe and Matthew Zimmer and David J. Kahler and Matthew Freeby and Wendy K. Chung and Charles A. Leduc and Robin Goland and Rudolph L Leibel and Dieter Egli},
  journal={Diabetes},
  year={2014},
  volume={63},
  pages={923 - 933}
}
Wolfram syndrome is an autosomal recessive disorder caused by mutations in WFS1 and is characterized by insulin-dependent diabetes mellitus, optic atrophy, and deafness. To investigate the cause of β-cell failure, we used induced pluripotent stem cells to create insulin-producing cells from individuals with Wolfram syndrome. WFS1-deficient β-cells showed increased levels of endoplasmic reticulum (ER) stress molecules and decreased insulin content. Upon exposure to experimental ER stress… Expand
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KCNH6 protects pancreatic β‐cells from endoplasmic reticulum stress and apoptosis
  • Jing-Kai Lu, Han Shen, +4 authors Jin-Kui Yang
  • Medicine, Chemistry
  • FASEB journal : official publication of the Federation of American Societies for Experimental Biology
  • 2020
TLDR
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TLDR
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