KCNJ10 and juvenile Huntington disease: For example, in vivo studies have shown that Kir4.1 loss leads to altered extracellular K+ dynamics in the hippocampus (Chever et al., 2010) and that restoration of Kir4.1 in striatal AS is sufficient to rescue extracellular K+ increase in a transgenic mouse model of Huntington’s disease (Tong et al., 2014).