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

Pediatr Nephrol. 2006 Sep;21(9):1231-6. doi: 10.1007/s00467-006-0106-6. Epub 2006 May 9.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Minor Histocompatibility Antigens
  • Protein-Serine-Threonine Kinases / physiology*
  • Pseudohypoaldosteronism / enzymology*
  • Pseudohypoaldosteronism / etiology*
  • WNK Lysine-Deficient Protein Kinase 1

Substances

  • Intracellular Signaling Peptides and Proteins
  • Minor Histocompatibility Antigens
  • Protein-Serine-Threonine Kinases
  • WNK Lysine-Deficient Protein Kinase 1
  • WNK1 protein, human
  • WNK4 protein, human