title: Induction of beta-galactosidase in beta-galactosidase-alpha-neuraminidase deficiency: effects of leupeptin and sucrose.
abstract: beta-Galactosidase was normalized by a serine-thiol protease inhibitor, leupeptin with concentration of 10 micrograms/ml in cultured skin fibroblasts from patients with beta-galactosidase-alpha-neuraminidase deficiency (beta-Gal-/Neu-). The induction of this enzyme was not observed in normal cells. Because the enzymic activity of cathepsin B1 increased significantly both in beta-Gal-/Neu- and normal cells by leupeptin loading, the restoration of beta-galactosidase in beta-Gal-/Neu- cells can not be explained by the theory that leupeptin inhibited intracellular degradation of beta-galactosidase molecules. The effects of leupeptin and sucrose on lysosomal hydrolase induction were compared.
mesh_terms: D002401:Cathepsin B; D002403:Cathepsins / Q000378:metabolism; D002460:Cell Line; D004790:Enzyme Induction; D005347:Fibroblasts; D005696:Galactosidases / Q000378:metabolism*; D006801:Humans; D006867:Hydrolases / Q000378:metabolism; D007787:Lactose Intolerance / Q000201:enzymology; D007976:Leupeptins / Q000494:pharmacology*; D008247:Lysosomes / Q000201:enzymology; D009439:Neuraminidase / Q000172:deficiency*; D009842:Oligopeptides / Q000494:pharmacology*; D013395:Sucrose / Q000494:pharmacology*; D001616:beta-Galactosidase / Q000378:metabolism*
chemical_list: D007976:Leupeptins; D009842:Oligopeptides; D013395:Sucrose; D006867:Hydrolases; D005696:Galactosidases; D009439:Neuraminidase; D001616:beta-Galactosidase; D002403:Cathepsins; D002401:Cathepsin B