Altered metabolic homeostasis in amyotrophic lateral sclerosis: mechanisms of energy imbalance and contribution to disease progression

Ioannides, Z. A., Ngo, S. T., Henderson, R. D., McCombe, P. A. and Steyn, F. J. (2016) Altered metabolic homeostasis in amyotrophic lateral sclerosis: mechanisms of energy imbalance and contribution to disease progression. Neurodegenerative Diseases, 15 5-6: 382-397. doi:10.1159/000446502


Author Ioannides, Z. A.
Ngo, S. T.
Henderson, R. D.
McCombe, P. A.
Steyn, F. J.
Title Altered metabolic homeostasis in amyotrophic lateral sclerosis: mechanisms of energy imbalance and contribution to disease progression
Journal name Neurodegenerative Diseases   Check publisher's open access policy
ISSN 1660-2862
1660-2854
Publication date 2016-09-01
Year available 2016
Sub-type Article (original research)
DOI 10.1159/000446502
Open Access Status Not yet assessed
Volume 15
Issue 5-6
Start page 382
End page 397
Total pages 16
Place of publication Basel, Switzerland
Publisher S. Karger
Collection year 2017
Language eng
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the death of motor neurones, which leads to paralysis and death in an average of 3 years following diagnosis. The cause of ALS is unknown, but there is substantial evidence that metabolic factors, including nutritional state and body weight, affect disease progression and survival. This review provides an overview of the characteristics of metabolic dysregulation in ALS focusing on mechanisms that lead to disrupted energy supply (at a whole-body and cellular level) and altered energy expenditure. We discuss how a decrease in energy supply occurs in parallel with an increase in energy demand and leads to a state of chronic energy deficit which has a negative impact on disease outcome in ALS. We conclude by presenting potential and tested strategies to compensate for, or correct this energy imbalance, and speculate on promising areas for further research.
Keyword Amyotrophic lateral sclerosis
Motor neurone diseaseĀ 
Metabolism
Energy expenditure
Nutrition
Neurometabolism
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

 
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