ArticleFrontiers in aging neuroscience2019
Reaction to Endoplasmic Reticulum Stress
Article in Frontiers in aging neuroscience, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 61 citations in OpenAlex.
- Dual targeting of the UPS and autophagy as a novel therapy for neurodegenerative proteinopathies.Frontiers in cellular neuroscience · 2026Review
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- Kratom use disorder and unfolded protein response: Evaluating their relationship in a case control study.PloS one · 2023Article
- Unfolded protein response IRE1/XBP1 signaling is required for healthy mammalian brain aging.The EMBO journal · 2022Article
- Autophagy and the hallmarks of aging.Ageing research reviews · 2021Review
- Circulating miR-181 is a prognostic biomarker for amyotrophic lateral sclerosis.Nature neuroscience · 2021Article
- Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations inFrontiers in molecular neuroscience · 2021Article
- Cerebrospinal Fluid Neurofilament Light Chain (NfL) Predicts Disease Aggressiveness in Amyotrophic Lateral Sclerosis: An Application of the D50 Disease Progression Model.Frontiers in neuroscience · 2021Article
- Endoplasmic Reticulum Stress and miRNA Impairment in Aging and Age-Related Diseases.Frontiers in aging · 2021Review
- Review
- Endoplasmic Reticulum Stress and Unfolded Protein Response in Neurodegenerative Diseases.International journal of molecular sciences · 2020Review
- Modelling disease course in amyotrophic lateral Sclerosis: pseudo-longitudinal insights from cross-sectional health-related quality of life data.Health and quality of life outcomes · 2020Article
- Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease.Disease models & mechanisms · 2020Article
- Article
- Poor emotional well-being is associated with rapid progression in amyotrophic lateral sclerosis.eNeurologicalSci · 2019Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Amyotrophic lateral sclerosis (ALS) is a multisystemic neurodegenerative disorder. Given that peripheral blood mononuclear cells (PBMCs) serve as a "window to the central nervous system" we aimed to answer whether endoplasmic reticulum (ER) stress in ALS-PBMCs is related to disease aggressiveness. We studied ER stress in the PBMCs of 49 patients with ALS and 31 age- and sex-matched healthy controls. The expression of a main ER stress marker, activating transcription factor 6 (ATF6), was significantly higher in ALS compared to controls, but did not correlate with age, disease severity, disease duration and disease progression rate. When ATF6 expression levels were plotted against relative D50 (rD50)-derived disease phases derived from the D50 ALS model, two distinct clusters of patients were observed: cluster 1, with progressively increasing ATF6 expression levels and cluster 2, which demonstrated stable ATF6 expression over the disease course. Individuals in the two clusters did not significantly differ in terms of ALS Functional Rating Scale-Revised (ALSFRS-R), disease aggressiveness, disease duration and subtype. However, patients with the increasing ATF6 level were significantly younger, indicating that aging processes might be related to ER stress in ALS. Our data suggest that the reaction to ER stress during disease course may be compromised in older patients with ALS.
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