It has been reported that the level of HMG-CoA reductase is reduced in ALS spinal cord grey matter, and that the SREBP2 expression level is lowered in ALS model mice with mutant SOD130, and also that Spinocerebellar Ataxia type 2 model mice harboring TDP-43 pathology exhibit concomitant cholesterol biogenesis suppression31. Here, SREBF2 is linked to spinocerebellar ataxia type 2.