Journal
ACTA NEUROPATHOLOGICA
Volume 135, Issue 4, Pages 489-509Publisher
SPRINGER
DOI: 10.1007/s00401-018-1835-x
Keywords
Amyotrophic lateral sclerosis; Energy metabolism; Neuron-glia metabolic coupling; Mitochondria; Metabolic dysfunction; Metabolic treatment
Categories
Funding
- KU Leuven (C1)
- KU Leuven (Opening the Future Fund)
- VIB
- Fund for Scientific Research Flanders (FWO-Vlaanderen)
- Belgian government [P7/16]
- Thierry Latran Foundation
- Association Belge contre les Maladies neuro-Musculaires (ABMM)
- ALS Liga Belgium (A Cure for ALS)
- FWO [1S60116N]
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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.
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