caprylic triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in als disease辛酸的甘油三酸酯作为一种新型治疗方法有效改善性能和减弱的症状由于运动神经元损失als疾病.pdfVIP

Caprylic Triglyceride as a Novel Therapeutic Approach to Effectively Improve the Performance and Attenuate the Symptoms Due to the Motor Neuron Loss in ALS Disease 1,2 1 1 1 1 1,2 Wei Zhao , Merina Varghese , Prashant Vempati , Anastasiya Dzhun , Alice Cheng , Jun Wang , 3 1 1 1,2 Dale Lange , Amanda Bilski , Irene Faravelli , Giulio Maria Pasinetti * 1 Department of Neurology, Mount Sinai School of Medicine, New York, New York, United States of America, 2 GRECC, James J Peters Veterans Affairs Medical Center, New York, New York, United States of America, 3 Hospital for Special Surgery, New York, New York, United States of America Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of motor neurons causing progressive muscle weakness, paralysis, and finally death. ALS patients suffer from asthenia and their progressive weakness negatively impacts quality of life, limiting their daily activities. They have impaired energy balance linked to lower activity of mitochondrial electron transport chain enzymes in ALS spinal cord, suggesting that improving mitochondrial function may present a therapeutic approach for ALS. When fed a ketogenic diet, the G93A ALS mouse shows a significant increase in serum ketones as well as a significantly slower progression of weakness and lower mortality rate. In this study, we treated SOD1-G93A mice with caprylic triglyceride, a medium chain triglyceride that is metabolized into ketone bodies and can serve as an alternate energy substrate for neuronal metabolism. Treatment w