Title |
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
|
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Published in |
Frontiers in Molecular Neuroscience, May 2017
|
DOI | 10.3389/fnmol.2017.00123 |
Pubmed ID | |
Authors |
Christopher P Webster, Emma F Smith, Pamela J Shaw, Kurt J De Vos |
Abstract |
Protein homeostasis (proteostasis), the correct balance between production and degradation of proteins, is essential for the health and survival of cells. Proteostasis requires an intricate network of protein quality control pathways (the proteostasis network) that work to prevent protein aggregation and maintain proteome health throughout the lifespan of the cell. Collapse of proteostasis has been implicated in the etiology of a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disorder. Here, we review the evidence linking dysfunctional proteostasis to the etiology of ALS and discuss how ALS-associated insults affect the proteostasis network. Finally, we discuss the potential therapeutic benefit of proteostasis network modulation in ALS. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United Kingdom | 5 | 36% |
United States | 3 | 21% |
Ireland | 1 | 7% |
Canada | 1 | 7% |
Unknown | 4 | 29% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 8 | 57% |
Scientists | 5 | 36% |
Practitioners (doctors, other healthcare professionals) | 1 | 7% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Australia | 1 | <1% |
Unknown | 177 | 99% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 41 | 23% |
Student > Bachelor | 26 | 15% |
Student > Master | 24 | 13% |
Researcher | 13 | 7% |
Student > Doctoral Student | 9 | 5% |
Other | 20 | 11% |
Unknown | 45 | 25% |
Readers by discipline | Count | As % |
---|---|---|
Biochemistry, Genetics and Molecular Biology | 44 | 25% |
Neuroscience | 37 | 21% |
Agricultural and Biological Sciences | 20 | 11% |
Medicine and Dentistry | 11 | 6% |
Pharmacology, Toxicology and Pharmaceutical Science | 5 | 3% |
Other | 14 | 8% |
Unknown | 47 | 26% |