期刊
NEURODEGENERATIVE DISEASES
卷 16, 期 5-6, 页码 382-397出版社
KARGER
DOI: 10.1159/000446502
关键词
Amyotrophic lateral sclerosis; Motor neurone disease; Metabolism; Neurometabolism; Nutrition; Energy expenditure
资金
- MNDRIA Bill Gole Fellowship
- Scott Sullivan MND Research Fellowship (Queensland Brain Institute)
- Scott Sullivan MND Research Fellowship (The Royal Brisbane and Worn en's Hospital Foundation)
- Scott Sullivan MND Research Fellowship (MND and Me Foundation)
- University of Queensland
- Royal Brisbane and Women's Hospital Foundation
- Australian and New Zealand Association of Neurologists (ANZAN) Education and Research Foundation/National Health and Medical Research Council (NHMRC) Postgraduate Scholarship
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. (C) 2016 S. Karger AG, Basel
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