Activating Compound | Comment | Organism | Structure |
---|---|---|---|
saposin C | optimal in vitro enzyme activity requires saposin C. Reduced saposin levels increase the instability of V394L or D409H GCases, the decreases leads to large accumulations of glucosylceramide in all tissues | Mus musculus |
Application | Comment | Organism |
---|---|---|
medicine | Gaucher disease mouse models | Mus musculus |
Protein Variants | Comment | Organism |
---|---|---|
D409H | homozygous, mice expressing low levels of prosaposin and saposins are backcrossed into mice with the point mutation D409H. In contrast to the mice with low levels of prosaposin and saposins the mutant mice with low levels of prosaposin and saposins display large numbers of engorged macrophages and nearly exclusive glucosylceramide accumulation in the liver, lung, spleen, thymus and brain | Mus musculus |
V394L | homozygous, mice expressing low levels of prosaposin and saposins are backcrossed into mice with the point mutation V394L. In contrast to the mice with low levels of prosaposin and saposins the mutant mice with low levels of prosaposin and saposins display large numbers of engorged macrophages and nearly exclusive glucosylceramide accumulation in the liver, lung, spleen, thymus and brain | Mus musculus |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
conduritol B epoxide | - |
Mus musculus |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
- |
- |
Synonyms | Comment | Organism |
---|---|---|
acid beta-glucosidase | - |
Mus musculus |
Gcase | - |
Mus musculus |