title: Multiple sulfatase deficiency: degradation of arylsulfatase A and B after endocytosis in fibroblasts.
abstract: Multiple sulfatase deficiency can be classified into group I with severe and group II with moderate deficiencies in sulfatases. In fibroblasts in both groups the stability of arylsulfatase A and of the 47000-Mr form of arylsulfatase B is decreased [F. Steckel, A. Hasilik & K. von Figura (1985) Eur. J. Biochem. 151, 141-145]. After endocytosis in control fibroblasts or those from multiple sulfatase deficiency, arylsulfatase A and B derived from the latter were subjected to enhanced degradation in both types of recipient cells. The degradation was closely linked in time to endocytosis. Whereas instability of arylsulfatase A derived from different cell lines from multiple sulfatase deficiency was comparable, a marked heterogeneity was observed for the instability of the 47000-Mr polypeptide of arylsulfatase B. Each of the cell lines from multiple sulfatase deficiency synthesized arylsulfatase A and B polypeptides with normal and with decreased stability. Treatment with benzyloxycarbonyl-Phe-Ala-CHN2, an inhibitor of cysteine proteinases, stabilized arylsulfatase A polypeptides and partially restored arylsulfatase A activity in group II fibroblasts. The inhibitor had no protective effect on the 47000-Mr polypeptide or the activity of arylsulfatase B. The bearing of these findings on the yet unknown primary defect in multiple sulfatase deficiency is discussed.
authors.forename: F
authors.initials: F
authors.lastname: Steckel
authors.forename: A
authors.initials: A
authors.lastname: Hasilik
authors.forename: K
authors.initials: K
authors.lastname: von Figura
mesh_terms: D002478:Cells, Cultured; D002553:Cerebroside-Sulfatase / Q000378:metabolism*; D002803:Chondro-4-Sulfatase / Q000378:metabolism*; D003978:Diazomethane / Q000031:analogs & derivatives / Q000494:pharmacology; D004705:Endocytosis; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D013429:Sulfatases / Q000172:deficiency* / Q000378:metabolism*
chemical_list: C024918:benzyloxycarbonylphenylalanylalanine diazomethyl ketone; D003978:Diazomethane; D013429:Sulfatases; D002553:Cerebroside-Sulfatase; D002803:Chondro-4-Sulfatase