X chromosome deletions detectable by flow cytometry in some patients with steroid sulphatase deficiency (X-linked ichthyosis)

Hum Genet. 1988 May;79(1):49-52. doi: 10.1007/BF00291709.

Abstract

The X chromosomes of individuals with isolated steroid sulphatase deficiency (X-linked ichthyosis) from ten families were studied by flow karyotype analysis. In four of the families, a small but significant reduction in the relative fluorescence of the X chromosome was detected consistent with a deletion ranging from 1.2%-3.4% of the X and amounting to a DNA loss of 1.9-5.2 million base pairs. In the remaining six families, three of which demonstrated a molecular deletion of the DNA sequence GMGX9 (DXS237), the relative fluorescence of the X chromosomes was indistinguishable from normal. The phenotypes of those with X deletions detectable by flow cytometry were similar to those of patients without such deletions.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chromosome Deletion*
  • DNA / genetics
  • Female
  • Flow Cytometry
  • Genetic Linkage*
  • Humans
  • Ichthyosis / enzymology
  • Ichthyosis / genetics*
  • Karyotyping
  • Male
  • Nucleic Acid Hybridization
  • Phenotype
  • Steryl-Sulfatase
  • Sulfatases / deficiency*
  • X Chromosome*

Substances

  • DNA
  • Sulfatases
  • Steryl-Sulfatase