Accordingly, in our HR patients, no neurological signs associated with grey matter astrocytic Kir4.1-linked dysfunctions were reported, such as those reported in autism-associated disorders50 or Huntington’s disease model.51 Similarly, we did not see anti-conformational e1-immunoreactivity on Cter-immunoreactive distal tubules in human kidney biopsies. Here, KCNJ10 is linked to Huntington disease.