KCNJ10 and juvenile Huntington disease: Direct evidence that loss of Kir4.1 alters neuronal function was recently demonstrated in a mouse model of Huntington’s disease, where AAV-mediated restoration of Kir4.1 expression specifically to astrocytes reduced the concentration of extracellular K+ ([K+]o), prolonged survival, and ameliorated motor abnormalities observed in these mice (Tong et al., 2014).