Article
Human N-acetylgalactosamine-4-sulphatase biosynthesis and maturation in normal, Maroteaux-Lamy and multiple-sulphatase-deficient fibroblasts.
The Biochemical journal - 1 Jun 1990
Taylor J A, Gibson G J, Brooks D A, Hopwood J J
Abstract excerpt
The biosynthesis and maturation of N-acetylgalactosamine-4-sulphatase (4-sulphatase) was studied in normal fibroblasts and in fibroblasts from patients with either mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome) or multiple sulphatase deficiency (MSD). Fibroblasts were incubated i...
Topics
- Adolescent
- Adult
- Cell Line
- Child, Preschool
- Electrophoresis, Polyacrylamide Gel
- Fibroblasts
- Humans
- Infant
- Mucopolysaccharidoses
- Mucopolysaccharidosis VI
- Mutation
- N-Acetylgalactosamine-4-Sulfatase
- Phenotype
- Sulfatases