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Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?

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 aggregatio...

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Autores principales: Webster, Christopher P., Smith, Emma F., Shaw, Pamela J., De Vos, Kurt J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411428/
https://www.ncbi.nlm.nih.gov/pubmed/28512398
http://dx.doi.org/10.3389/fnmol.2017.00123
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author Webster, Christopher P.
Smith, Emma F.
Shaw, Pamela J.
De Vos, Kurt J.
author_facet Webster, Christopher P.
Smith, Emma F.
Shaw, Pamela J.
De Vos, Kurt J.
author_sort Webster, Christopher P.
collection PubMed
description 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.
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spelling pubmed-54114282017-05-16 Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities? Webster, Christopher P. Smith, Emma F. Shaw, Pamela J. De Vos, Kurt J. Front Mol Neurosci Neuroscience 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. Frontiers Media S.A. 2017-05-02 /pmc/articles/PMC5411428/ /pubmed/28512398 http://dx.doi.org/10.3389/fnmol.2017.00123 Text en Copyright © 2017 Webster, Smith, Shaw and De Vos. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Webster, Christopher P.
Smith, Emma F.
Shaw, Pamela J.
De Vos, Kurt J.
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title_full Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title_fullStr Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title_full_unstemmed Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title_short Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
title_sort protein homeostasis in amyotrophic lateral sclerosis: therapeutic opportunities?
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411428/
https://www.ncbi.nlm.nih.gov/pubmed/28512398
http://dx.doi.org/10.3389/fnmol.2017.00123
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