Activating Compound | Comment | Organism | Structure |
---|---|---|---|
N-(n-butyl)deoxynojirimycin | other names are miglustat and Zavesca, about 20% increase of wild type enzyme activity at 0.0025 and 0.005 mM | Homo sapiens | |
N-(n-nonyl) deoxynojirimycin | 1.3-1.4fold increase in activity is observed for the wild type enzyme at 0.02 mM | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expressed in COS-7 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
D409H | the mutant shows about 17% activity compared to the wild type enzyme | Homo sapiens |
E326K | the mutant shows about 30% activity compared to the wild type enzyme | Homo sapiens |
G202R | the mutant shows about 25% activity compared to the wild type enzyme | Homo sapiens |
G377S | the mutant shows about 25% activity compared to the wild type enzyme | Homo sapiens |
H255Q | the mutant shows about 75% activity compared to the wild type enzyme | Homo sapiens |
H255Q/D409H | the mutant shows about 20% activity compared to the wild type enzyme | Homo sapiens |
I402T | the mutant shows about 19% activity compared to the wild type enzyme | Homo sapiens |
L444P | the mutant shows about 3% activity compared to the wild type enzyme | Homo sapiens |
N188S | the mutant shows about 55% activity compared to the wild type enzyme | Homo sapiens |
N188S/E326K | the mutant shows about 10% activity compared to the wild type enzyme | Homo sapiens |
N370S | the mutant shows about 7% activity compared to the wild type enzyme | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
glucosylceramide + H2O | Homo sapiens | - |
beta-D-glucose + ceramide | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl-beta-D-glucoside + H2O | - |
Homo sapiens | 4-methylumbelliferone + beta-D-glucose | - |
? | |
glucosylceramide + H2O | - |
Homo sapiens | beta-D-glucose + ceramide | - |
? |
Synonyms | Comment | Organism |
---|---|---|
GBA | - |
Homo sapiens |
glucocerebrosidase | - |
Homo sapiens |