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dc.contributor.authorMatus, Soledaden_US
dc.contributor.authorValenzuela, Vicenteen_US
dc.contributor.authorMedinas, Danilo B.en_US
dc.contributor.authorHetz, Claudioen_US
dc.date.accessioned2014-03-11T10:16:09Z
dc.date.issued2013en_US
dc.identifier.citationMatus, Soledad, Vicente Valenzuela, Danilo B. Medinas, and Claudio Hetz. 2013. “ER Dysfunction and Protein Folding Stress in ALS.” International Journal of Cell Biology 2013 (1): 674751. doi:10.1155/2013/674751. http://dx.doi.org/10.1155/2013/674751.en
dc.identifier.issn1687-8876en
dc.identifier.urihttp://nrs.harvard.edu/urn-3:HUL.InstRepos:11879275
dc.description.abstractAmyotrophic lateral sclerosis (ALS) is the most frequent paralytic disease in adults. Most ALS cases are considered sporadic with no clear genetic component. The disruption of protein homeostasis due to chronic stress responses at the endoplasmic reticulum (ER) and the accumulation of abnormal protein inclusions are extensively described in ALS mouse models and patient-derived tissue. Recent studies using pharmacological and genetic manipulation of the unfolded protein response (UPR), an adaptive reaction against ER stress, have demonstrated a complex involvement of the pathway in experimental models of ALS. In addition, quantitative changes in ER stress-responsive chaperones in body fluids have been proposed as possible biomarkers to monitor the disease progression. Here we review most recent advances attributing a causal role of ER stress in ALS.en
dc.language.isoen_USen
dc.publisherHindawi Publishing Corporationen
dc.relation.isversionofdoi:10.1155/2013/674751en
dc.relation.hasversionhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3845333/pdf/en
dash.licenseLAAen_US
dc.titleER Dysfunction and Protein Folding Stress in ALSen
dc.typeJournal Articleen_US
dc.description.versionVersion of Recorden
dc.relation.journalInternational Journal of Cell Biologyen
dash.depositing.authorHetz, Claudioen_US
dc.date.available2014-03-11T10:16:09Z
dc.identifier.doi10.1155/2013/674751*
dash.contributor.affiliatedHetz, Claudio


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