ER Dysfunction and Protein Folding Stress in ALS
Joint Authors
Matus, Soledad
Hetz, Claudio
Medinas, Danilo B.
Valenzuela, Vicente
Source
International Journal of Cell Biology
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-11-13
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
Amyotrophic 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.
American Psychological Association (APA)
Matus, Soledad& Valenzuela, Vicente& Medinas, Danilo B.& Hetz, Claudio. 2013. ER Dysfunction and Protein Folding Stress in ALS. International Journal of Cell Biology،Vol. 2013, no. 2013, pp.1-12.
https://search.emarefa.net/detail/BIM-489527
Modern Language Association (MLA)
Matus, Soledad…[et al.]. ER Dysfunction and Protein Folding Stress in ALS. International Journal of Cell Biology No. 2013 (2013), pp.1-12.
https://search.emarefa.net/detail/BIM-489527
American Medical Association (AMA)
Matus, Soledad& Valenzuela, Vicente& Medinas, Danilo B.& Hetz, Claudio. ER Dysfunction and Protein Folding Stress in ALS. International Journal of Cell Biology. 2013. Vol. 2013, no. 2013, pp.1-12.
https://search.emarefa.net/detail/BIM-489527
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-489527