Up-regulated GS expression was reported in vascular dementia and ALS (Tumani et al., 1999), whereas reduced GS activity was found in hepatic encephalopathy, spinocerebellar atrophy, schizophrenia and epilepsy (Lavoie et al., 1987; Smith et al., 1991; Kish et al., 1994; Le Prince et al., 1995; Burbaeva et al., 2003; van der Hel et al., 2005; Steffek et al., 2008). Here, GLUL is linked to Spinocerebellar atrophy.