Rapid molecular diagnosis of erythropoietic protoporphyria among Swiss patients

Clin Chem Lab Med. 1998 Oct;36(10):763-5. doi: 10.1515/CCLM.1998.135.

Abstract

Erythropoietic protoporphyria (EPP) is an autosomal dominant inherited disorder with incomplete penetrance. It is caused by partial deficiency of ferrochelatase, the last enzyme in the heme biosynthetic pathway. Measurement of protoporphyrin concentrations in red cells and feces, although sufficient for diagnosis of symptomatic EPP patients, fails to detect asymptomatic gene carriers. We have developed a molecular diagnostic procedure for rapid and reliable screening of five known mutations in the ferrochelatase gene among Swiss EPP patients in a single denaturing gradient gel electrophoresis (DGGE) gel.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Electrophoresis, Polyacrylamide Gel / methods
  • Genetic Carrier Screening
  • Genetic Testing
  • Humans
  • Mutation
  • Porphyria, Hepatoerythropoietic / diagnosis*
  • Porphyria, Hepatoerythropoietic / ethnology
  • Porphyria, Hepatoerythropoietic / genetics
  • Switzerland / ethnology