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

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41 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1231-1236
Number of pages6
JournalPediatric Nephrology
Issue number9
StatePublished - Sep 2006


  • Chloride shunt
  • ENaC
  • Maxi-K
  • NCC
  • NCCT
  • ROMK
  • Sodium-chloride co-transporter

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology


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