| Title : Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors - Suzuki_1981_J.Biochem_90_271 |
| Author(s) : Suzuki Y , Sakuraba H , Hayashi K , Suzuki K , Imahori K |
| Ref : J Biochem , 90 :271 , 1981 |
|
Abstract :
Beta-Galactosidase was partially restored by protease inhibitors, leupeptin, chymostatin and E-64 in cultured fibroblasts from three patients with beta-galactosidase-neuraminidase deficiency. Pepstatin did not activate this enzyme. Neuraminidase was not affected by any of these compounds in the culture medium. It was concluded that the activating effect was produced by a specific inhibition of thiol proteases. |
| PubMedSearch : Suzuki_1981_J.Biochem_90_271 |
| PubMedID: 6793566 |
| Gene_locus related to this paper: human-CTSA |
| Gene_locus | human-CTSA |
| Disease | Galactosialidosis |
Suzuki Y, Sakuraba H, Hayashi K, Suzuki K, Imahori K (1981)
Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors
J Biochem
90 :271
Suzuki Y, Sakuraba H, Hayashi K, Suzuki K, Imahori K (1981)
J Biochem
90 :271