Rapid degradation of steroid sulfatase in multiple sulfatase deficiency.
Pulse labeling followed by SDS-PAGE electrophoresis of immunoprecipitated [35S]methionine-labeled steroid sulfatase (STS) gave a single band of molecular weight 65,000 daltons. After a chase period of 18 hours the material appeared as molecular weight approximately 64,000. No labeled STS could be detected in fibroblasts from individuals with STS deficient X-linked ichthyosis. Pulse-chase labeling of normal and multiple sulfatase deficiency (MSD) fibroblasts showed a normal rate of synthesis of STS in MSD during a 3 hour pulse but during the chase the STS of MSD cells disappeared with a half-life of 4 to 6 hours until approximately 25% of the material remained after 24 hr. STS of normal cells had a half-life of 6 days. The material produced in MSD cells had the same molecular size as normal and had the same amount of endoglycosidase sensitive carbohydrate as normal. The defect in MSD thus seems to result in degradation after the addition of N-linked oligosaccharides.
Horwitz
A L
AL
Warshawsky
L
L
King
J
J
Burns
G
G
D000118:Acetylglucosaminidase; D050260:Carbohydrate Metabolism; D011232:Chemical Precipitation; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007120:Immunochemistry; D066298:In Vitro Techniques; D017038:Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase; D008715:Methionine / Q000378:metabolism; D008970:Molecular Weight; D010779:Photofluorography; D043266:Steryl-Sulfatase; D013429:Sulfatases / Q000096:biosynthesis / Q000172:deficiency* / Q000378:metabolism*
D008715:Methionine; D013429:Sulfatases; D043266:Steryl-Sulfatase; D000118:Acetylglucosaminidase; D017038:Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase