Title |
Altered Metabolic Homeostasis in Amyotrophic Lateral Sclerosis: Mechanisms of Energy Imbalance and Contribution to Disease Progression
|
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Published in |
Neurodegenerative Diseases, July 2016
|
DOI | 10.1159/000446502 |
Pubmed ID | |
Authors |
Zara A. Ioannides, Shyuan T. Ngo, Robert D. Henderson, Pamela A. McCombe, Frederik J. Steyn |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Australia | 2 | 50% |
Unknown | 2 | 50% |
Demographic breakdown
Type | Count | As % |
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Scientists | 2 | 50% |
Members of the public | 2 | 50% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Italy | 1 | 2% |
Unknown | 43 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 7 | 16% |
Student > Master | 6 | 14% |
Researcher | 5 | 11% |
Student > Bachelor | 4 | 9% |
Professor | 4 | 9% |
Other | 5 | 11% |
Unknown | 13 | 30% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 10 | 23% |
Neuroscience | 5 | 11% |
Biochemistry, Genetics and Molecular Biology | 3 | 7% |
Agricultural and Biological Sciences | 2 | 5% |
Pharmacology, Toxicology and Pharmaceutical Science | 2 | 5% |
Other | 6 | 14% |
Unknown | 16 | 36% |