Gitelman syndrome: genetic and expression analysis of the thiazide-sensitive sodium-chloride transporter in blood cells

Nephrol Dial Transplant. 2006 Jan;21(1):217-20. doi: 10.1093/ndt/gfi093. Epub 2005 Oct 12.

Abstract

Gitelman syndrome is caused by mutations of the SLC12A3 gene, which encodes the thiazide-sensitive NaCl transporter NCCT. Although several mutations causing Gitelman syndrome have been described, their molecular consequences have been rarely studied. We report a patient with Gitelman syndrome due to a mutation in the GT donor splicing site of intron 9. The analysis of RNA from peripheral blood cells showed a complete deletion of exon 9. This case report confirms the feasibility of using readily accessible blood cells to study the expression of the SLC12A3 gene, a procedure that may facilitate further studies of the functional genomics of Gitelman syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Alkalosis / blood
  • Alkalosis / diagnosis
  • Alkalosis / drug therapy
  • Alkalosis / genetics*
  • Base Sequence
  • Cells, Cultured
  • Erythrocytes
  • Gene Expression Regulation
  • Genetic Predisposition to Disease*
  • Humans
  • Hypokalemia / diagnosis
  • Hypokalemia / drug therapy
  • Hypokalemia / genetics*
  • Magnesium / blood*
  • Male
  • Molecular Sequence Data
  • Mutation*
  • Prognosis
  • RNA / analysis
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sodium Chloride Symporters / genetics*
  • Syndrome
  • Thiazides / therapeutic use

Substances

  • Sodium Chloride Symporters
  • Thiazides
  • RNA
  • Magnesium