Article
Analysis of N-acetylgalactosamine-4-sulfatase protein and kinetics in mucopolysaccharidosis type VI patients.
American journal of human genetics - 1 Apr 1991
Brooks D A, McCourt P A, Gibson G J, Ashton L J, Shutter M, Hopwood J J
Abstract excerpt
A sensitive and specific, monoclonal antibody-based immunoquantification assay has facilitated determination of the N-acetylgalactosamine-4-sulfatase (4-sulfatase) protein content in cultured fibroblasts from normal controls and mucopolysaccharidosis type VI (MPS VI) patients. The assay enabled the quantification of 4-sulfatase protein by using a panel of seven monoclonal antibodies and has shown that fibroblasts...
Topics
- Antibodies, Monoclonal
- Cells, Cultured
- Chondro-4-Sulfatase
- Dermatan Sulfate
- Enzyme-Linked Immunosorbent Assay
- Fibroblasts
- Humans
- Kinetics
- Mucopolysaccharidoses
- Phenotype
- Reagent Kits, Diagnostic
