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Role of with-no-lysine [K] kinases in the pathogenesis of Gordon’s syndrome
Journal article   Peer reviewed

Role of with-no-lysine [K] kinases in the pathogenesis of Gordon’s syndrome

Jian Xie, Leonard Craig, Melanie Cobb and Chou-Long Huang
Pediatric nephrology (Berlin, West), Vol.21(9), pp.1231-1236
09/2006
DOI: 10.1007/s00467-006-0106-6
PMID: 16683163

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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.
Pediatrics NCCT Medicine & Public Health Chloride shunt Maxi-K Sodium–chloride co-transporter ENaC ROMK NCC

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