More than 75% reduction of glutamate transporter 1 (GLT1, also known as excitatory amino acid transporter 2, EAAT2, or solute carrier family 1 member 2, SLC1A2) immunoreactivity was observed in mouse models of AxD and astrocytes in hippocampal CA1 region of human patients show variable to complete loss of immunostaining for EAAT2 (Tian et al., 2010b). Here, SLC1A2 is linked to Alexander disease.