[SPECIFICITY OF HELIX POMATIA SULFATASES. STUDY OF THE KINETICS OF HYDROLYSIS OF SULFATED ESTERS].
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Steroidsulfatase and arylsulfatase C were determined in fibroblasts and/or leukocytes of patients affected with different types of ichthyosis. Of the 21 patients studied, 11 showed clinical characteristics of X-linked ichthyosis (XLI) and a deficiency of these 2 enzymatic activities. Patients affected with other types of ichthyosis showed no enzymatic deficiency. In XLI families diagnosis of heterozygotes was performed by enzymatic measurements in the 5 patients' mothers studied. In 2 families enzymatic activities were studied in patients' sisters. The validity of these different enzymatic measurements is discussed.
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Arylsulfatases B1 and B2 isolated from human brain were introduced into neonatal type MSD fibroblast culture. Arylsulfatase B2 was linearly taken up into MSD fibroblasts with respect to time and the dose of enzyme, whereas arylsulfatase B1 enzyme was practically not taken up into cells. Uptake of arylsulfatase B2 was inhibited in the presence of mannose 6-phosphate. These data suggest that the biological functional difference between arylsulfatases B1 and B2 is due to the presence of a phosphorylated residue acting as a recognition marker on one of the enzymes.
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