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Cited by 24Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsHereditary Neurological Disorders

Abnormal energy metabolism in ALS: a key player?

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Burg, Thibaut, Van Den Bosch, Ludo·2023·Current Opinion in Neurology
DOI 10.1097/wco.0000000000001164PMC PMC10328537
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Abstract

Purpose of the review: Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease of the motor system due to the selective and progressive degeneration of both upper and lower motor neurons. Disturbances in energy homeostasis were repeatedly associated with the ALS pathogenesis and appear early during the disease process. In this review, we highlight recent work demonstrating the crucial role of energy metabolism in ALS and discuss its potential clinical relevance. Recent findings: The alteration of various metabolic pathways contributes to the heterogeneity of the clinical phenotype of ALS. Recent work showed that different ALS mutations selectively impact these pathways and translate to the disease phenotypes in patients and disease models. Strikingly, a growing number of studies point towards an early, even presymptomatic, contribution of abnormal energy homeostasis to the ALS pathogenesis. Advances in metabolomics generated valuable tools to study altered metabolic pathways, to test their therapeutic potential, and to develop personalized medicine. Importantly, recent preclinical studies and clinical trials demonstrated that targeting energy metabolism is a promising therapeutic approach. Summary: Abnormal energy metabolism is a key player in ALS pathogenesis, emerging as a source of potential disease biomarkers and therapeutic targets.

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Cited by

Sorted by citations

Authors
Burg, T., & Van Den Bosch, L.
Affiliations
  • VIB-KU Leuven Center for Brain & Disease Research (BE)
  • Departement of Neuroscience, Experimental Neurology, Leuven Brain Institute and KU Leuven
ORCID
Burg, Thibaut
Venue
Current Opinion in Neurology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2023-06-19
Volume
36
Issue
4
Pages
338-345
ISSN
  • 1350-7540 (ISSN-L)
  • 1473-6551
DOI
10.1097/wco.0000000000001164
PMC
PMC10328537
OpenAlex
W4381309123
Cited by
24
References
6
Citations by year
2026: 9 · 2025: 8 · 2024: 5 · 2023: 2
FWCI
0.98
Type
journal article
Language
en
Open access
Licence
Locations
  • Current Opinion in Neurology (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • Lirias (KU Leuven) (submittedVersion, other-oa)
Funders
  • Muscular Dystrophy Association
  • Association Belge contre les Maladies Neuro-Musculaires
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
amyotrophic lateral sclerosis, energy metabolism, energy homeostasis, ALS pathogenesis, motor neuron degeneration, metabolic pathways, ALS mutations, clinical phenotype heterogeneity, presymptomatic disease, metabolomics, disease biomarkers, therapeutic target, personalized medicine
MeSH terms
Amyotrophic Lateral Sclerosis, Energy Metabolism, Humans, Motor Neurons, Neurodegenerative Diseases, Precision Medicine

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