Corticostriatal dysfunction and glutamate transporter 1 (GLT1) in Huntington's disease: interactions between neurons and astrocytes

AM Estrada-Sánchez, GV Rebec - Basal ganglia, 2012 - Elsevier
Huntington's disease (HD) is a fatally inherited neurodegenerative disorder caused by an
expanded glutamine repeat in the N-terminal region of the huntingtin (HTT) protein. The
result is a progressively worsening triad of cognitive, emotional, and motor alterations that
typically begin in adulthood and end in death 10–20 years later. Autopsy of HD patients
indicates massive cell loss in the striatum and its main source of input, the cerebral cortex.
Further studies of HD patients and transgenic animal models of HD indicate that …