Reduction in GLT-1 mRNA level is well reported in various brain disorders like ischemia, amyotrophic lateral sclerosis, Alzheimer's disease and Huntington's disease (Torp et al., 1995; Sims and Robinson, 1999; Gegelashvili et al., 2001) with subsequent consequences of secondary neuronal cell death, excessive activation of glutamate receptors, abnormal neuronal activity and ensuing excitotoxic degeneration (Rothstein et al., 1996; Tanaka et al., 1997). The gene discussed is SLC1A2; the disease is juvenile Huntington disease.