Application | Comment | Organism |
---|---|---|
medicine | Gaucher disease, sphingolipoidose | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expression in Sf9 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
D358E | no activity on glucosylceramide and 4-methylumbelliferyl-beta-D-glucopyranoside | Homo sapiens |
N370S | Gaucher disease type I mutation | Homo sapiens |
R120Q | Gaucher disease type I mutation, inactive | Homo sapiens |
T43K | decreased activity on glucosylceramides | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
1-deoxynojirimycin | - |
Homo sapiens | |
6-bromo-conduritol-beta-epoxide | - |
Homo sapiens | |
castanospermine | - |
Homo sapiens | |
glucosylsphingosine | - |
Homo sapiens | |
N-decylglycosylamine | - |
Homo sapiens | |
N-dodecylglycosylamine | - |
Homo sapiens | |
N-hexadecylglycosylamine | - |
Homo sapiens | |
N-hexyl-D-glucosylsphingosine | - |
Homo sapiens | |
N-octadecylglycosylamine | - |
Homo sapiens | |
N-tetradecylglycosylamine | - |
Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
glucocerebroside + H2O | Homo sapiens | - |
glucose + ceramide | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
normal and Gaucher disease enzyme | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | skin fibroblasts in culture | Homo sapiens | - |
spleen | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl-beta-D-glucopyranoside + H2O | - |
Homo sapiens | methylumbelliferone + glucose | - |
? | |
glucocerebroside + H2O | - |
Homo sapiens | glucose + ceramide | - |
? |