N-acetyl-β-hexosaminidase secreted by cultured skin fibroblasts from normal individuals, or from patients with mucopolysaccharidoses, is effectively captured by fibroblasts derived from patients with Sandhoff disease. On the other hand, N-acetyl-β-hexosaminidase from several other sources (including placenta, urine, and secretions of fibroblasts from patients with mucolipidosis II or III) is taken up to a much lesser extent. The uptake of the normal fibroblast hydrolase can be abolished by periodate oxidation under conditions that do not affect its catalytic activity, stability, or binding to Concanavalin A - Sepharose. These results suggest that uptake into fibroblasts requires a specific marker on the enzyme.
|Number of pages||7|
|Journal||Biochemical and Biophysical Research Communications|
|State||Published - Mar 15 1974|