the turnover of lysosomal beta-galactosidase was studied in fibroblast cultures from patients with gm1-gangliosidosis and combined beta-galactosidase and neuraminidase deficiency , which had 5-10 % residual beta-galactosidase activity . beta-galactosidase was specifically inactivated with the suicide substrate beta-d-galactopyranosylmethyl-p-nitro-phenyltriazene ( beta-gal-mnt ) and from the subsequent restoration of enzyme activity in cell cultures turnover times were calculated .
C0016030
fibroblast
C0007634
cell