Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by progressive muscular atrophy and pyramidal deficit, due to the degeneration of upper and lower motor neurons, with an average survival of approximately 3-5 years from symptom onset. The pathogenesis of the disease is not yet clear. Riluzole and edaravone are the only FDA-approved drugs to treat ALS. Riluzole prolongs life by only a few months and edaravone improves patient functionality scores in a subset of patients. Due to the unavailability of effective drugs, there is an urgent need for new treatment modalities in ALS. A growing body of evidence shows dysregulated energy metabolism in ALS patients and models. Studies also found that eating behaviors and metabolic changes correlate with the change of structure and function of the hypothalamic melanocortin system. In addition, enhancement of mitochondrial function in ATP production and/or utilization could bring some kinds of benefit to the ALS patients or models. Here, we review disturbances in energy metabolism in ALS patients and models, and provide an overview of new energy metabolism approaches in ALS.

Amyotrophic lateral sclerosis; Energy metabolism; Review