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

Molecular Genetics and Metabolism

Volumn 104, Issue 4, 2011, Pages 566-573

  Shimada, Yohta ^a   Kobayashi, Hiroshi ^a   Kawagoe, Shiho ^a   Aoki, Katsuhiko ^a   Kaneshiro, Eiko ^a   Shimizu, Hiromi ^a   Eto, Yoshikatsu ^a   Ida, Hiroyuki ^a   Ohashi, Toya ^a  

^a JIKEI UNIVERSITY SCHOOL OF MEDICINE   (Japan)

Author keywords

Autophagy; Endoplasmic reticulum stress; NB DNJ; P38; Pharmacological chaperone; Pompe disease

Indexed keywords

4 (4 FLUOROPHENYL) 2 (4 METHYLSULFINYLPHENYL) 5 (4 PYRIDYL)IMIDAZOLE; ALPHA GLUCOSIDASE; ENZYME INHIBITOR; MITOGEN ACTIVATED PROTEIN KINASE P38; N BUTYL DEOXYNOJIRIMYCIN; PROTEASOME; UNCLASSIFIED DRUG;

ARTICLE; AUTOPHAGY; DRUG MECHANISM; ENDOPLASMIC RETICULUM STRESS; ENZYME PHOSPHORYLATION; ENZYME REPLACEMENT; FIBROBLAST; GENE MUTATION; GLYCOGEN STORAGE DISEASE TYPE 2; HUMAN; HUMAN CELL; MUTANT; MUTATION; PRIORITY JOURNAL; PROTEIN FOLDING; SIGNAL TRANSDUCTION;

1-DEOXYNOJIRIMYCIN; ALPHA-GLUCOSIDASES; AUTOPHAGY; CELLS, CULTURED; DNA MUTATIONAL ANALYSIS; ENDOPLASMIC RETICULUM STRESS; ENZYME ACTIVATION; ENZYME ACTIVATORS; FIBROBLASTS; GLYCOGEN STORAGE DISEASE TYPE II; MAP KINASE SIGNALING SYSTEM; P38 MITOGEN-ACTIVATED PROTEIN KINASES; PHOSPHOPROTEINS; PHOSPHORYLATION; POINT MUTATION; PROTEIN FOLDING; PROTEIN ISOFORMS; UNFOLDED PROTEIN RESPONSE;

EID: 82255179378     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.09.005     Document Type: Article

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