Sulfatase activity of keratinizing tissues in X-linked ichthyosis

Pediatr Res. 1980 Dec;14(12):1347-8. doi: 10.1203/00006450-198012000-00015.

Abstract

Arylsulfatases A and B were measured in the stratum corneum of four normal controls and two individuals with sex-linked ichthyosis. For arylsulfatase A, the mean delta optical density/hr/mg protein value was 1.6 for controls and 2.0 for patients, whereas for arylsulfatase B values of 1.5 for controls and 1.4 for patients were observed. Assay of arylsulfatase C in the callus of four normal controls showed a mean delta optical density/hr/100 mg callus of 0.63, whereas no or trace activity was detected in callus from four patients with x-linked ichthyosis. The assay of steroid sulfatase is best for studying microsomal sulfatase activity. Table 1 shows the activity of this enzyme in nails, callus, and hair bulbs from controls and patients with x-linked ichthyosis. No steroid sulfatase could be demonstrated in patients with x-linked ichthyosis. The values in normal controls and obligate heterozygotes are compared in Table 2. The mean value of the two groups is statistically different with P less than or equal to 0.05 using the Student t test.

MeSH terms

  • Arylsulfatases / deficiency
  • Arylsulfatases / metabolism*
  • Female
  • Genetic Linkage
  • Hair / enzymology*
  • Heterozygote
  • Humans
  • Ichthyosis / enzymology*
  • Ichthyosis / genetics
  • Nails / enzymology*
  • Sex Chromosome Aberrations / genetics
  • Sulfatases / metabolism*
  • X Chromosome

Substances

  • Sulfatases
  • Arylsulfatases