Synthesis and stability of steroid sulfatase in fibroblasts from multiple sulfatase deficiency.
Multiple sulfatase deficiency is a lysosomal storage disorder, which can be divided into group I with severe and group II with moderate deficiencies in sulfatases. Antibodies raised against steroid sulfatase purified from human placenta were used to follow the biosynthesis and stability of this enzyme in multiple sulfatase-deficiency fibroblasts. Fibroblasts from both groups synthesized steroid sulfatase of apparently normal size and stability, while the apparent rate of enzyme synthesis and catalytic properties of steroid sulfatase were affected to a variable extent. Cell lines were observed, that synthesized normal amounts of steroid-sulfatase polypeptides, which were catalytically inactive, as well as cell lines that synthesized diminished amounts of catalytically active steroid sulfatase.
Conary
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JT
Institut für Biochemie II der Universität Göttingen.
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D002460:Cell Line; D002478:Cells, Cultured; D004795:Enzyme Stability; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007700:Kinetics; D043266:Steryl-Sulfatase; D013429:Sulfatases / Q000096:biosynthesis / Q000172:deficiency* / Q000378:metabolism*
D013429:Sulfatases; D043266:Steryl-Sulfatase