The importance of Ca2+ overload in ALS has been extensively discussed, and while studies have shown changes in MCU/MICU1/MICU2 expression in ALS (Fuchs et al., 2013; Mühling et al., 2014; Dafinca et al., 2020), studies targeting mitochondrial Ca2+ uptake and release mechanisms have been limited (Tadić et al., 2019). Here, MICU1 is linked to amyotrophic lateral sclerosis.