Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

Purpose of Review Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease for which there is no cure and treatments are at best palliative. Several genes have been linked to ALS, which highlight defects in multiple cellular processes including RNA processing, proteostasis, and...

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Bibliographic Details
Published in:Current genetic medicine reports 2017-06, Vol.5 (2), p.108-114
Main Authors: Joardar, Archi, Manzo, Ernesto, Zarnescu, Daniela C.
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Dyslipidemia
Fatty acids
Glucose tolerance
Glycolysis
Internal Medicine
Intolerance
Medicine
Medicine & Public Health
Metabolism
Mitochondria
Motor neurons
Motor task performance
Muscles
Neurogenetics and Psychiatric Genetics
Neurogenetics and Psychiatric Genetics (M Hiltunen and DR Marenda
Oxidation
RNA processing
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Summary:Purpose of Review Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease for which there is no cure and treatments are at best palliative. Several genes have been linked to ALS, which highlight defects in multiple cellular processes including RNA processing, proteostasis, and metabolism. Clinical observations have identified glucose intolerance and dyslipidemia as key features of ALS; however, the causes of these metabolic alterations remain elusive. Recent Findings Recent studies reveal that motor neurons and muscle cells may undergo cell type-specific metabolic changes that lead to utilization of alternate fuels. For example, ALS patients’ muscles exhibit reduced glycolysis and increased reliance on fatty acids. In contrast, ALS motor neurons contain damaged mitochondria and exhibit impaired lipid beta oxidation, potentially leading to increased glycolysis as a compensatory mechanism. Summary These findings highlight the complexities of metabolic alterations in ALS and provide new opportunities for designing therapeutic strategies based on restoring cellular energetics.
ISSN:2167-4876
2167-4876
DOI:10.1007/s40142-017-0123-8