Novel variant transthyretin gene (Ser50 to Ile) in familial cardiac amyloidosis

Biochem Biophys Res Commun. 1992 Aug 31;187(1):460-6. doi: 10.1016/s0006-291x(05)81516-5.

Abstract

We detected a point mutation in the transthyretin (TTR) gene in a patient with familial cardiac amyloidosis by using PCR-DCP (DNA conformation polymorphism) analysis that is based on the diversity in electrophoretic mobility of single-stranded DNAs and/or heteroduplex DNAs in PCR products. The PCR products of the transthyretin gene were denatured in the presence of formamide and electrophoresed in a non-denaturing polyacrylamide gel to detect an electrophoretic change due to a sequence variation. An unusual DNA fragment was visualized by silver staining in the PCR products of the exon 3 from the patient. Subsequent sequencing analysis revealed a T to A transversion and led to a replacement of Ser by Ile at codon 50 of the TTR gene.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloidosis / genetics*
  • Base Sequence
  • Cardiomyopathies / genetics*
  • Cloning, Molecular
  • DNA / chemistry
  • DNA / genetics
  • Electrophoresis, Polyacrylamide Gel
  • Humans
  • Isoleucine / genetics
  • Japan
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Mutation*
  • Nucleic Acid Conformation
  • Nucleic Acid Hybridization
  • Polymerase Chain Reaction
  • Polymorphism, Genetic
  • Prealbumin / genetics*
  • Serine / genetics

Substances

  • Prealbumin
  • Isoleucine
  • Serine
  • DNA