Endoplasmic reticulum stress induces autophagy through activation of p38 MAPK in fibroblasts from Pompe disease patients carrying c.546G>T mutation.

@article{Shimada2011EndoplasmicRS,
  title={Endoplasmic reticulum stress induces autophagy through activation of p38 MAPK in fibroblasts from Pompe disease patients carrying c.546G>T mutation.},
  author={Yohta Shimada and Hiroshi Kobayashi and Shiho Kawagoe and Katsuhiko Aoki and Eiko Kaneshiro and Hiromi Shimizu and Yoshikatsu Eto and Hiroyuki Ida and Toya Ohashi},
  journal={Molecular genetics and metabolism},
  year={2011},
  volume={104 4},
  pages={566-73}
}
Pompe disease (glycogen storage disease type II) is an autosomal recessive myopathic disorder arising from the deficiency of lysosomal acid α-glucosidase (GAA). Activation of autophagy is a key pathophysiological feature in skeletal muscle fibers and fibroblasts from patients with Pompe disease. The accumulation of autophagic vacuoles has been shown to interfere with the efficacy of enzyme replacement therapy with recombinant human GAA. However, the induction mechanism of autophagy in Pompe… CONTINUE READING
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