Altered Metabolism in Motor Neuron Diseases: Mechanism and Potential Therapeutic Target

卷:12

Review

关键词: AMYOTROPHIC-LATERAL-SCLEROSIS;    HEREDITARY SPASTIC PARAPLEGIA;    SPINAL MUSCULAR-ATROPHY;    LIMB MUSCLE INVOLVEMENT;    MITOCHONDRIAL DYSFUNCTION;    MOUSE MODEL;    SKELETAL-MUSCLE;    OXIDATIVE-PHOSPHORYLATION;    KENNEDYS DISEASE;    SMN GENE;   

DOI  :  10.3390/cells12111536

来源: SCIE

【 摘 要 】

Motor Neuron Diseases (MND) are neurological disorders characterized by a loss of varying motor neurons resulting in decreased physical capabilities. Current research is focused on hindering disease progression by determining causes of motor neuron death. Metabolic malfunction has been proposed as a promising topic when targeting motor neuron loss. Alterations in metabolism have also been noted at the neuromuscular junction (NMJ) and skeletal muscle tissue, emphasizing the importance of a cohesive system. Finding metabolism changes consistent throughout both neurons and skeletal muscle tissue could pose as a target for therapeutic intervention. This review will focus on metabolic deficits reported in MNDs and propose potential therapeutic targets for future intervention.