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Energy metabolism in ALS: an underappreciated opportunity?
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive and fatal neurodegenerative disorder that primarily affects motor neurons. Despite our increased understanding of the genetic factors contributing to ALS, no effective treatment is available. A growing body of evidence shows disturban...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5978930/ https://www.ncbi.nlm.nih.gov/pubmed/29549424 http://dx.doi.org/10.1007/s00401-018-1835-x |
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author | Vandoorne, Tijs De Bock, Katrien Van Den Bosch, Ludo |
author_facet | Vandoorne, Tijs De Bock, Katrien Van Den Bosch, Ludo |
author_sort | Vandoorne, Tijs |
collection | PubMed |
description | Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive and fatal neurodegenerative disorder that primarily affects motor neurons. Despite our increased understanding of the genetic factors contributing to ALS, no effective treatment is available. A growing body of evidence shows disturbances in energy metabolism in ALS. Moreover, the remarkable vulnerability of motor neurons to ATP depletion has become increasingly clear. Here, we review metabolic alterations present in ALS patients and models, discuss the selective vulnerability of motor neurons to energetic stress, and provide an overview of tested and emerging metabolic approaches to treat ALS. We believe that a further understanding of the metabolic biology of ALS can lead to the identification of novel therapeutic targets. |
format | Online Article Text |
id | pubmed-5978930 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-59789302018-06-21 Energy metabolism in ALS: an underappreciated opportunity? Vandoorne, Tijs De Bock, Katrien Van Den Bosch, Ludo Acta Neuropathol Review Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive and fatal neurodegenerative disorder that primarily affects motor neurons. Despite our increased understanding of the genetic factors contributing to ALS, no effective treatment is available. A growing body of evidence shows disturbances in energy metabolism in ALS. Moreover, the remarkable vulnerability of motor neurons to ATP depletion has become increasingly clear. Here, we review metabolic alterations present in ALS patients and models, discuss the selective vulnerability of motor neurons to energetic stress, and provide an overview of tested and emerging metabolic approaches to treat ALS. We believe that a further understanding of the metabolic biology of ALS can lead to the identification of novel therapeutic targets. Springer Berlin Heidelberg 2018-03-16 2018 /pmc/articles/PMC5978930/ /pubmed/29549424 http://dx.doi.org/10.1007/s00401-018-1835-x Text en © The Author(s) 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Review Vandoorne, Tijs De Bock, Katrien Van Den Bosch, Ludo Energy metabolism in ALS: an underappreciated opportunity? |
title | Energy metabolism in ALS: an underappreciated opportunity? |
title_full | Energy metabolism in ALS: an underappreciated opportunity? |
title_fullStr | Energy metabolism in ALS: an underappreciated opportunity? |
title_full_unstemmed | Energy metabolism in ALS: an underappreciated opportunity? |
title_short | Energy metabolism in ALS: an underappreciated opportunity? |
title_sort | energy metabolism in als: an underappreciated opportunity? |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5978930/ https://www.ncbi.nlm.nih.gov/pubmed/29549424 http://dx.doi.org/10.1007/s00401-018-1835-x |
work_keys_str_mv | AT vandoornetijs energymetabolisminalsanunderappreciatedopportunity AT debockkatrien energymetabolisminalsanunderappreciatedopportunity AT vandenboschludo energymetabolisminalsanunderappreciatedopportunity |