Alternative fuels in epilepsy and amyotrophic lateral sclerosis

Tefera, Tesfaye W. , Tan, Kah Ni, McDonald, Tanya S.  and Borges, Karin (2016) Alternative fuels in epilepsy and amyotrophic lateral sclerosis. Neurochemical Research, 42 6: 1-11. doi:10.1007/s11064-016-2106-7

Author Tefera, Tesfaye W. 
Tan, Kah Ni
McDonald, Tanya S. 
Borges, Karin
Title Alternative fuels in epilepsy and amyotrophic lateral sclerosis
Journal name Neurochemical Research   Check publisher's open access policy
ISSN 1573-6903
Publication date 2016-11-21
Year available 2017
Sub-type Article (original research)
DOI 10.1007/s11064-016-2106-7
Open Access Status Not yet assessed
Volume 42
Issue 6
Start page 1
End page 11
Total pages 11
Place of publication New York, NY, United States
Publisher Springer New York LLC
Language eng
Subject 1303 Biochemistry
2804 Cellular and Molecular Neuroscience
Abstract This review summarises the recent findings on metabolic treatments for epilepsy and Amyotrophic Lateral Sclerosis (ALS) in honour of Professor Ursula Sonnewald. The metabolic impairments in rodent models of these disorders as well as affected patients are being discussed. In both epilepsy and ALS, there are defects in glucose uptake and reduced tricarboxylic acid (TCA) cycling, at least in part due to reduced amounts of C4 TCA cycle intermediates. In addition there are impairments in glycolysis in ALS. A reduction in glucose uptake can be addressed by providing the brain with alternative fuels, such as ketones or medium-chain triglycerides. As anaplerotic fuels, such as the triglyceride of heptanoate, triheptanoin, refill the TCA cycle C4/C5 intermediate pool that is deficient, they are ideal to boost TCA cycling and thus the oxidative metabolism of all fuels.
Keyword ALS
Ketogenic diet
Medium chain triglyceride
Tricarboxylic acid cycle
Q-Index Code C1
Q-Index Status Provisional Code
Grant ID 1044007
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
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School of Biomedical Sciences Publications
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