摘要
Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that is characterized by the selectivedegeneration of upper motor neurons and lower spinal motor neurons, resulting in the progressive paralysis of allvoluntary 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 ofprogression of disease. This review summarizes current research surrounding the impact of impaired metabolicphysiology in ALS. We extend ideas to consider prospects that lie ahead in terms of how metabolic alterations mayimpact the selective degeneration of neurons in ALS and how targeting of adenosine triphosphate-sensitive potassium(K_(ATP)) channels may represent a promising approach for obtaining neuroprotection in ALS.
基金
The authors dedicate this manuscript in memory of Mr Bob Delaney
STN acknowledges the support of the Queensland Brain Institute,the Royal Brisbane and Women’s Hospital,the MND and Me Foundation
the School of Biomedical Sciences,and a Bob Delaney MND research grant from the MNDRIA
FJS acknowledges the support of the University of Queensland Centre for Clinical Research,the School of Biomedical Sciences,and a Cunningham Collaboration research grant from the MNDRIA.
