Journal article

AMPK Signalling and Defective Energy Metabolism in Amyotrophic Lateral Sclerosis

Nirma D Perera, Bradley J Turner



Amyotrophic lateral sclerosis (ALS) is caused by selective loss of upper and lower motor neurons by complex mechanisms that are incompletely understood. Motor neurons are large, highly polarised and excitable cells with unusually high energetic demands to maintain resting membrane potential and propagate action potentials. This leads to higher ATP consumption and mitochondrial metabolism in motor neurons relative to other cells. Here, we review increasing evidence that defective energy metabolism and homeostasis contributes to selective vulnerability and degeneration of motor neurons in ALS. Firstly, we provide a brief overview of major energetic pathways in the CNS, including glycolysis, ox..

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Awarded by Australian National Health and Medical Research Council

Funding Acknowledgements

This work was supported by the Australian National Health and Medical Research Council (Project Grant 1008910), Stafford Fox Medical Research Foundation, MND Research Institute of Australia (Ted Dimmick Memorial MND Research Grant), Bethlehem Griffiths Research Foundation, Cavalier Courage MND Research Grant and the Victorian Government's Operational Infrastructure Support Grant. N.P. is supported by an Australian Postgraduate Award Scholarship.