TSPO is expressed in glial cells, neurons, and endothelialandependymal cells and localized in the outer mitochondrial membranein enriched steroidogenic regions. Itsfunction has been widely associated with steroidogenesis, but also linked to other functions such as mitochondrialbioenergetics,, redox mechanisms,, and neuroinflammation. Enhanced TSPOlevels in glial cells and neurons were reported in pathological conditions,including ALS, whereas under physiological conditions, TSPO is poorlyexpressed. Thus, TSPO is an interestingtarget for ALS. This evidence concerns the gene TSPO and amyotrophic lateral sclerosis.