title: Role of with-no-lysine [K] kinases in the pathogenesis of Gordon's syndrome.
abstract: Gordon's syndrome, also known as pseudohypoaldosteronism type II (PHA II) or familial hypertension with hyperkalemia, is an autosomal-dominant disease characterized by hypertension, hyperkalemia, hyperchloremic metabolic acidosis, and normal glomerular filtration rate. Recent positional cloning has linked mutations of WNK1 and WNK4 to Gordon's syndrome. With-no-lysine [K] (WNK) kinases are a new family of large serine-threonine protein kinases with an atypical placement of the catalytic lysine. Here, we review the pathogenesis of PHA II based on current understanding of the actions of WNK1 and WNK4 on Na+ and K+ handling in the renal distal tubule.
authors: Department of Medicine, Room J5-104, MC-8856, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-8856, USA.
authors: Jian
authors: J
authors: Xie
authors: Leonard
authors: L
authors: Craig
authors: Melanie H
authors: MH
authors: Cobb
authors: Chou-Long
authors: CL
authors: Huang