Constitutively active SPAK causes hyperkalemia by activating NCC and remodeling distal tubules

PR Grimm, R Coleman, E Delpire… - Journal of the American …, 2017 - journals.lww.com
Aberrant activation of with no lysine (WNK) kinases causes familial hyperkalemic
hypertension (FHHt). Thiazide diuretics treat the disease, fostering the view that
hyperactivation of the thiazide-sensitive sodium-chloride cotransporter (NCC) in the distal
convoluted tubule (DCT) is solely responsible. However, aberrant signaling in the
aldosterone-sensitive distal nephron (ASDN) and inhibition of the potassium-excretory renal
outer medullary potassium (ROMK) channel have also been implicated. To test these ideas …