Protein S mRNA in patients with protein S deficiency

Thromb Haemost. 1995 May;73(5):746-9.

Abstract

A protein S gene polymorphism, detectable by restriction analysis (BstXI) of amplified exonic sequences (exon 15), was studied in seven Italian families with protein S deficiency. In the 17 individuals heterozygous for the polymorphism the study was extended to platelet mRNA through reverse transcription, amplification and densitometric analysis. mRNA produced by the putative defective protein S genes was absent in three families and reduced to a different extent (as expressed by altered allelic ratios) in four families. The allelic ratios helped to distinguish total protein S deficiency (type I) for free protein S deficiency (type IIa) in families with equivocal phenotypes. This study indicates that the study of platelet mRNA, in association with phenotypic analysis based upon protein S assays in plasma, helps to classify patients with protein S deficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alleles
  • Base Sequence
  • Blood Platelets / chemistry
  • Deoxyribonucleases, Type II Site-Specific
  • Female
  • Genotype
  • Humans
  • Male
  • Molecular Sequence Data
  • Pedigree
  • Phenotype
  • Polymorphism, Restriction Fragment Length*
  • Protein S / genetics*
  • Protein S Deficiency / classification
  • Protein S Deficiency / diagnosis
  • Protein S Deficiency / genetics*
  • RNA, Messenger / genetics*
  • RNA, Messenger / isolation & purification

Substances

  • Protein S
  • RNA, Messenger
  • CCANNNNNNTGG-specific type II deoxyribonucleases
  • Deoxyribonucleases, Type II Site-Specific

Grants and funding