MTDH and juvenile Huntington disease: Recently, the multifunctional oncogene, astrocyte elevated gene-1 (AEG-1), was shown to dysregulate glutamate clearance by excitatory amino acid transporter 2 downregulation and HIV-associated neuroinflammation via upregulation of nuclear factor-κB (NF-κB) pathway in astrocytes.20,21 AEG-1 was also elevated in a Huntington’s disease model along with UPR markers such as BiP, C/EBP homologous protein (CHOP), and regulator of ribosome synthesis 1.22 These studies suggest the involvement of AEG-1 in ER stress responses during neurological diseases, including HAND.