Genetic screening of 104 patients with congenitally malformed hearts revealed a fresh mutation of GATA4 in those with atrial septal defects

Cardiol Young. 2009 Sep;19(5):482-5. doi: 10.1017/S1047951109990813. Epub 2009 Aug 13.

Abstract

We analysed the GATA binding protein 4 gene, or GATA4, along with the NK2 transcription factor related, locus 5 gene, or NKX2.5, to determine their genetic contribution to 104 sporadic patients in Indonesia with congenitally malformed hearts, 76 cases having atrial septal defect and 28 tetralogy of Fallot. We found only 1 novel mutation of GATA4 in those with atrial septal defects. Analysis of the genetic background of the parents of the patient showed for the first time that a new mutation of GATA4 can cause sporadic atrial septal defects. We failed to discover any other mutations of either the GATA4 or NKX2-5 genes, supporting the marked genetic heterogeneity of human congenital cardiac defects.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child, Preschool
  • GATA4 Transcription Factor / genetics*
  • Genetic Testing*
  • Heart Septal Defects, Atrial / genetics*
  • Humans
  • Male
  • Mutation*
  • Pedigree
  • Tetralogy of Fallot / genetics*

Substances

  • GATA4 Transcription Factor
  • GATA4 protein, human