Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10833830 | Molecular Genetics and Metabolism | 2011 | 8 Pages |
Abstract
⺠Mutant GAA was degraded by proteasome in Pompe fibroblasts with c.546G>T mutation. ⺠Both activation of ER stress and autophagy was observed in the patient fibroblasts. ⺠NB-DNJ led to attenuation of not only ER stress, but also autophagy in patient cells. ⺠Increased level of phosphorylated p38 MAPK in patient cells were decreased by NB-DNJ. ⺠Autophagy in patient cells was also negatively regulated by p38 inhibitor SB203580.
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Authors
Yohta Shimada, Hiroshi Kobayashi, Shiho Kawagoe, Katsuhiko Aoki, Eiko Kaneshiro, Hiromi Shimizu, Yoshikatsu Eto, Hiroyuki Ida, Toya Ohashi,