Sulphatase (Steroid Sulphatase in white cells) (Also known as arylsulphatase C)

General Information

Steroid sulphatase, or arylsulphatase C, is an enzyme which primarily hydrolyses sulphate from a variety of steroids, but has specificity for a range of aromatic sulphates. It is present in all tissues but is particularly abundant in placental tissue, where its role is to liberate steroids from sulphate stores. The gene for steroid sulphatase is located near the end of the short arm of the X-chromosome and escapes inactivation. Hence females tend to have levels of activity approximately twice that of males. X-linked ichthyosis is a skin disorder due to a defect in the steroid sulphatase gene, but only usually occurs in males and unless there are adjacent genes affected due to a major deletion, the only major problem that occurs is a skin disorder. Multiple sulphatase deficiency is an autosomal recessive disorder, and is much more severe. Many sulphatases are deficient including arylsulphatase A, B, C, and sulphatases involved in heparan and keratan sulphate catabolism. Hence these patients often have symptoms similar to that seen in defects of all of these enzymes. Arylsulphatase C is usually assayed when arylsulphatase A deficiency has been detected, in order to differentiate between metachromatic leucodystrophy and multiple sulphatase deficiency.