Types I and III Gaucher disease in Poland: incidence of the most common mutations and phenotypic manifestations

Eur J Hum Genet. 1996;4(6):334-7. doi: 10.1159/000472228.

Abstract

Gaucher disease caused by hereditary deficiency of beta-glucocerebrosidase is the most prevalent lysosomal storage disease. The incidence of the 5 commonest mutations was estimated in the Polish Gaucher disease population. A trial to establish genotype/phenotype correlations was performed. A relatively high frequency of type III disease can be stated in the studied Polish Gaucher patients. The most frequent mutation was L444P, followed by the N370S mutation. A distinct correlation between genotype and phenotype was observed in the studied group of Polish patients with Gaucher disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Gaucher Disease / epidemiology
  • Gaucher Disease / genetics*
  • Gaucher Disease / physiopathology
  • Genetic Markers
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Mutation
  • Phenotype
  • Poland / epidemiology

Substances

  • Genetic Markers