SLC1A2 and juvenile Huntington disease: As described above, because EAAT2 is the predominant L-Glu transporter in the frontal cortex, defective EAAT2 transport leads to excitotoxic neuronal death [16,17,18,19], which has been identified as one of the pathological changes in Huntington’s disease [19,20], amyotrophic lateral sclerosis (ALS) [17,21], and schizophrenia [18].