| http://purl.uniprot.org/citations/26266407 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26266407 | http://www.w3.org/2000/01/rdf-schema#comment | "Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder that affects upper and lower motor neurons. Since motor neurons target skeletal muscles, the maintenance system of muscles is disturbed in ALS; however, the mechanism by which this occurs is unknown. In the present study, we investigated the effects of ALS-associated P56S-vesicle-associated membrane protein-associated protein B (VAPB) (P56S-VAPB) on the IRE1-XBP1 pathway, which is involved in the unfolded protein response (UPR) of the mouse myoblast cell line (C2C12 cells). Experiments with C2C12 cells transfected with wild-type wt-VAPB and P56S-VAPB expression vectors showed reduced myotube formation and aberrant myonuclear position in cells expressing P56S-VAPB. Activity of the IRE1-XBP1 pathway in the cells visualized with the ERAI system revealed that the pathway was disrupted in cells expressing P56S-VAPB, whereas the IRE1-XBP1 pathway activity was enhanced in the differentiation process of normal C2C12 cells. These results suggest that disruption of the IRE1-XBP1 pathway is a cause for the reduced myotube formation in P56S-VAPB-expressing cells. The expression level of the VAPB protein has been reported to be reduced in the neurons of patients with ALS. Therefore, it is expected that the IRE1-XBP1 pathway is also impaired in muscle tissues of patients with ALS, which causes a disturbance in the muscle maintenance system."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.org/dc/terms/identifier | "doi:10.3390/ijms160818628"xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Yamada K."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Tokutake Y."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Tsuchiya M."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Yonekura S."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Obayashi Y."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/author | "Ohata M."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/name | "Int J Mol Sci"xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/pages | "18628-18641"xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/title | "ALS-Linked P56S-VAPB Mutation Impairs the Formation of Multinuclear Myotube in C2C12 Cells."xsd:string |
| http://purl.uniprot.org/citations/26266407 | http://purl.uniprot.org/core/volume | "16"xsd:string |
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