Interestingly, though mutations in KCNJ10 have not been linked to neurodegenerative disorders, analysis of animal models of AD, amyotrophic lateral sclerosis (ALS), and Huntington’s disease suggest that downregulation of Kir4.1 in astrocytes may contribute to disease onset and/or progression (Kaiser et al., 2006; Wilcock et al., 2009; Tong et al., 2014). The gene discussed is KCNJ10; the disease is juvenile Huntington disease.