GYPE and Huntington disease: Pallidal neurons in human HD show upregulation of GABAA receptors in response to loss of inhibitory input as striatal projection neurons die, with the pattern of upregulation in globus pallidus (i.e., GPe vs GPi) reflecting the pattern of striatal projection neuron loss—that is upregulation in GPe occurs at earlier grades than upregulation in GPi due to the greater loss of iSPNs projecting to GPe than dSPNs projecting to GPi (Glass, Dragunow, & Faull, 2000; Reiner ‐ Dragatsis, & Dietrich, 2011).