Keratin 14 gene point mutation in the Köbner and Dowling-Meara types of epidermolysis bullosa simplex as detected by the PASA method

Arch Dermatol Res. 1995;287(2):142-5. doi: 10.1007/BF01262322.

Abstract

Recent advances in molecular biology have enabled the association of epidermolysis bullosa simplex (EBS) with point mutations of keratin 14 and/or keratin 5 genes to be established. We describe here the detection of point mutations in genomic DNA from formalin-fixed and paraffin-embedded sections from five cases of epidermolysis bullosa using the PCR amplification of specific alleles (PASA) method. In two of four cases of Köbner-type EBS a point mutation of helix 2b (384 Leu-Pro) was detected and in one case of Dowling-Meara-type EBS a mutation in helix 1a (125 Arg-Cys) was detected. The results of this study are consistent with previous reports and they demonstrate that the PASA method is a rapid and reproducible method for the detection of single-base changes and small deletions.

Publication types

  • Comparative Study

MeSH terms

  • Alleles*
  • Amino Acid Sequence
  • Base Sequence
  • DNA Primers
  • Epidermolysis Bullosa Simplex / classification*
  • Epidermolysis Bullosa Simplex / genetics*
  • Humans
  • Keratins / chemistry
  • Keratins / genetics*
  • Molecular Sequence Data
  • Point Mutation*
  • Polymerase Chain Reaction / methods*
  • Protein Structure, Secondary
  • Templates, Genetic

Substances

  • DNA Primers
  • Keratins