SOD1 and amyotrophic lateral sclerosis: In line with the effects of SOD1 on FAO observed in our laboratory, recent experiments in a TDP-43 Drosophila model of ALS revealed that TDP-43 pathology alters the transcription of crucial components of the carnitine shuttle, leading to a significant reduction in lipid beta oxidation.148 Although it is unclear what underlines this distinctive malign activity of TDP-43, dietary supplementation with medium-chain fatty acids, which independently cross the mitochondrial membrane, mitigated the carnitine deficit and rescued motor neuron survival.