SOD1 and amyotrophic lateral sclerosis: In vitro and in vivo studies using ALS model systems of superoxide dismutase 1 (SOD1)-related familial ALS have provided first evidence of metabolic dysfunction in ALS astrocytes, particularly in the transporter responsible for the efflux of lactate43, dysfunctional astrocytic mitochondria44, and lower levels of intracellular lactate upon exposure of astrocytes to glutamate45.