This accumulation is strongly linked to the selective loss or dysfunction of the major astrocytic glutamate transporter, EAAT2, observed in the motor cortex and spinal cord of ALS patients and in transgenic ALS mouse (SOD1 mutant) models (Rothstein et al., 1995; Howland et al., 2002). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.