Open access
Date
2018-04Type
- Journal Article
Abstract
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. Show more
Permanent link
https://doi.org/10.3929/ethz-b-000254305Publication status
publishedExternal links
Journal / series
Acta NeuropathologicaVolume
Pages / Article No.
Publisher
SpringerSubject
Amyotrophic lateral sclerosis; Energy metabolism; Neuron-glia metabolic coupling; Mitochondria; Metabolic dysfunction; Metabolic treatmentOrganisational unit
09560 - De Bock, Katrien / De Bock, Katrien
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