The interplay between metabolic homeostasis and neurodegeneration: insights into the neurometabolic nature of amyotrophic lateral sclerosis

Ngo, S. T. and Steyn, F. J. (2015) The interplay between metabolic homeostasis and neurodegeneration: insights into the neurometabolic nature of amyotrophic lateral sclerosis. Cell Regeneration, 4 5: 1-14. doi:10.1186/s13619-015-0019-6


Author Ngo, S. T.
Steyn, F. J.
Title The interplay between metabolic homeostasis and neurodegeneration: insights into the neurometabolic nature of amyotrophic lateral sclerosis
Journal name Cell Regeneration   Check publisher's open access policy
ISSN 2045-9769
Publication date 2015-08-27
Year available 2015
Sub-type Article (original research)
DOI 10.1186/s13619-015-0019-6
Open Access Status DOI
Volume 4
Issue 5
Start page 1
End page 14
Total pages 14
Place of publication London, United Kingdom
Publisher BioMed Central
Language eng
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that is characterized by the selective degeneration of upper motor neurons and lower spinal motor neurons, resulting in the progressive paralysis of all voluntary muscles. Approximately 10 % of ALS cases are linked to known genetic mutations, with the remaining 90 % of cases being sporadic. While the primary pathology in ALS is the selective death of upper and lower motor neurons, numerous studies indicate that an imbalance in whole body and/or cellular metabolism influences the rate of progression of disease. This review summarizes current research surrounding the impact of impaired metabolic physiology in ALS. We extend ideas to consider prospects that lie ahead in terms of how metabolic alterations may impact the selective degeneration of neurons in ALS and how targeting of adenosine triphosphate-sensitive potassium (KATP) channels may represent a promising approach for obtaining neuroprotection in ALS.
Formatted abstract
Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that is characterized by the selective degeneration of upper motor neurons and lower spinal motor neurons, resulting in the progressive paralysis of all voluntary muscles. Approximately 10 % of ALS cases are linked to known genetic mutations, with the remaining 90 % of cases being sporadic. While the primary pathology in ALS is the selective death of upper and lower motor neurons, numerous studies indicate that an imbalance in whole body and/or cellular metabolism influences the rate of progression of disease. This review summarizes current research surrounding the impact of impaired metabolic physiology in ALS. We extend ideas to consider prospects that lie ahead in terms of how metabolic alterations may impact the selective degeneration of neurons in ALS and how targeting of adenosine triphosphate-sensitive potassium (KATP) channels may represent a promising approach for obtaining neuroprotection in ALS.
Keyword Amyotrophic lateral sclerosis
Metabolism
Neurometabolism
Hyperexcitability
Ion channels
KATP channels
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

 
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Created: Wed, 09 Dec 2015, 23:06:56 EST by Susan Day on behalf of Queensland Brain Institute