title: The mitochondrial DNA mutations associated with cardiac arrhythmia investigated in an LQTS family.
abstract: OBJECTIVE
  As mitochondrial oxidative stress is probably entailed in ATP production, a candidate modifier factor for the long QT syndrome (LQTS) could be mitochondrial DNA (mtDNA). It has been notified that ion channels' activities in cardiomyocytes are sensitive to the ATP level.
  
  
  METHODS
  The sample of the research was an Iranian family with LQTS for mutations by PCR-SSCP and DNA sequencing. The study searched about 40% of the entire mitochondrial genome in the family.
  
  
  RESULTS
  Four novel mutations that lead to an amino acid substitution and two mutations in mitochondrial tRNA have been informed in this study. A Statistically significant correlation (r = 0.737) between QTc (ms) and the age of LQTS patients has been reported.
  
  
  CONCLUSIONS
  The research data show that these mitochondrial mutations, in a family with LQTS, might be the responsible mitochondrial that defect and increase the gravity of LQTS.
authors.affiliation: Department of Biology, Yazd University, Yazd, Iran.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Khatami
authors.affiliation: Department of Biology, Yazd University, Yazd, Iran.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Mehdi Heidari
authors.affiliation: Department of Medical Genetics, National Institute for Genetic Engineering and Biotechnology, Tehran, Iran.
authors.forename: Massoud
authors.initials: M
authors.lastname: Houshmand
keywords: Arrhythmia; Long QT syndrome; Mitochondrial DNA; Mutation, SSCP