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Review

Altered Metabolic Homeostasis in Amyotrophic Lateral Sclerosis: Mechanisms of Energy Imbalance and Contribution to Disease Progression

Ioannides Z.A.a, c · Ngo S.T.a,d,e · Henderson R.D.b, c · McCombe P.A.a-c · Steyn F.J.a, d

Author affiliations

aUniversity of Queensland Centre for Clinical Research, and bSchool of Medicine, The University of Queensland, and cDepartment of Neurology, Royal Brisbane and Women's Hospital, Herston, Qld., and dSchool of Biomedical Sciences, and eQueensland Brain Institute, The University of Queensland, St Lucia, Qld., Australia

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Neurodegener Dis 2016;16:382-397

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Article / Publication Details

First-Page Preview
Abstract of Review

Received: April 01, 2016
Accepted: April 27, 2016
Published online: July 12, 2016
Issue release date: September 2016

Number of Print Pages: 16
Number of Figures: 2
Number of Tables: 2

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: https://www.karger.com/NDD

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.

© 2016 S. Karger AG, Basel


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Article / Publication Details

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Abstract of Review

Received: April 01, 2016
Accepted: April 27, 2016
Published online: July 12, 2016
Issue release date: September 2016

Number of Print Pages: 16
Number of Figures: 2
Number of Tables: 2

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

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