Table 5.

Kidney channelopathies.

Disease	Gene (protein)	Pharmacotherapy and mechanisms of action	Pharmacological perspectives
Bartter’s syndrome (BS) including types II–IV	CLCNKB (ClC-Kb), BSDN (Barttin), ROMK1 (Kir1.1)	Symptomatic therapy aims at restoring electrolyte balance: – Potassium-sparing diuretics such as spironolactone/eplerenone and amiloride restore serum K+ concentrations – Indomethacin, a COX inhibitor, reduces PGE2 production – Potassium and magnesium supplements restore electrolyte balance	– Development of selective channel openers through a pharmacogenetic approach – Development of pharmacological chaperones
Dent disease type 1	CLCN5 (ClC-5)	Symptomatic therapy aims at restoring electrolyte balance: – Thiazide diuretics ameliorate hypercalciuria – High citrate and fluid intake control hypercalciuria – Vitamin D counteracts rickets in clinical cases with additional bone disease	– Development of CLC-5 activators – Development of pharmacological chaperones
EAST/SESAME syndrome	KCNJ11 (Kir4.1)	Symptomatic therapy aims at restoring electrolyte balance and remitting seizures: – Antiepileptic drugs – Indometacin – Oral potassium