Résumé
Aim Amyotrophic lateral sclerosis (Alii) is a neurodegenerative disease caused by the selective loss of upper and lower motor neurons.Its pathogenesis is complex and not yet fully understood.Accumulating evidences show that energy metabolism defieits and homeostasis imbalance lead to selective degeneration of motor neurons in ALS.Adenosine phosphate-activated protein kinase ( AMPK) and silent information regulator-1 (SIRT1) are known to sense energy metabolism at the cellular level, playing an essential part in regulating the energy homeostasis and autophagy by activating downstream pathways.AMPK activates SIRT1 to activate the deaeetylation and activation of downstream genes involved in energy metabolism such as peroxisome proliferator-aetivated receptor 7 ( PPAR-y) and eoaetivator la ( PGC-la) , thereby increasing the level of mitochondrial biosynthesis.Therefore, the mechanism by which AMPK/SIRT1 participates in the regulation of energy metabolism is highly likely to be a potential target for ALS treatment.Here we first review the important role of energy metabolism defects and mitochondrial abnormalities in the pathogenesis of ALS, and briefly address the therapeutic potential of AMPK/SIRT1/PGC-1 a pathway as a treatment target in ALS, and how targeted regulation of this signaling pathway makes it an effective therapeutic strategy for ALS.