Galactosialidosis (β-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of β-galactosidase molecules

Abstract β-Galactosidase was restored by serine-thiol protease inhibitors in cultured skin fibroblasts from patients with galactosialidosis. Neuraminidase was not influenced by these compounds. Cathepsin B activity in patients' fibroblasts was moderately increased as compared to that in control cells. Some serine-thiol protease like cathepsin B may play a role in the degradation of β-galactosidase molecules in tissues of patients with this disease.

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