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

Research output: Contribution to journalArticle

40 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1231-1236
Number of pages6
JournalPediatric Nephrology
Volume21
Issue number9
DOIs
StatePublished - Sep 2006

Fingerprint

Pseudohypoaldosteronism
Hyperkalemia
Lysine
Phosphotransferases
Distal Kidney Tubule
Hypertension
Protein-Serine-Threonine Kinases
Acidosis
Glomerular Filtration Rate
Organism Cloning
Mutation
Gordon syndrome

Keywords

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

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

Role of with-no-lysine [K] kinases in the pathogenesis of Gordon's syndrome. / Xie, Jian; Craig, Leonard; Cobb, Melanie H.; Huang, Chou Long.

In: Pediatric Nephrology, Vol. 21, No. 9, 09.2006, p. 1231-1236.

Research output: Contribution to journalArticle

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