Activating Compound | Comment | Organism | Structure |
---|---|---|---|
1-deoxygalactonojirimycin | 3.8fold increase at 0.02 mM after 5 days | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
mutants expressed in COS-7 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A156V | reduced activity | Homo sapiens |
A20P | reduced activity | Homo sapiens |
A97V | reduced activity | Homo sapiens |
E59K | reduced activity | Homo sapiens |
E66Q | reduced activity | Homo sapiens |
F113L | reduced activity | Homo sapiens |
I91T | reduced activity | Homo sapiens |
L166V | reduced activity | Homo sapiens |
M296I | reduced activity | Homo sapiens |
M296V | reduced activity | Homo sapiens |
M72V | reduced activity | Homo sapiens |
N215S | reduced activity | Homo sapiens |
Q279E | reduced activity | Homo sapiens |
R112H | reduced activity | Homo sapiens |
R301Q | reduced activity | Homo sapiens |
R356W | reduced activity | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
1.8 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme Q279E, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
1.9 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme R301Q, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme R356W, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.4 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme M296V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.6 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme L166V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.7 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme M296I, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.8 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme A20P, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.8 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme N215S, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
2.8 | - |
4-methylumbelliferyl-alpha-D-galactoside | wild type enzyme, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.1 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme E66Q, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.2 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme M72V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.4 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme A156V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.4 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme I91T, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.6 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme F113L, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
3.7 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme A97V, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
5.8 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme R112H, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens | |
16.3 | - |
4-methylumbelliferyl-alpha-D-galactoside | mutant enzyme E59K, in 0.1 M sodium citrate buffer (pH 4.6) at 37°C | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
46000 | - |
mature lysosomal enzyme form, SDS-PAGE | Homo sapiens |
50000 | - |
ER and Golgi enzyme forms, SDS-PAGE | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
lymphoblast | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl-alpha-D-galactoside + H2O | - |
Homo sapiens | 4-methylumbelliferol + alpha-D-galactose | - |
? |
Synonyms | Comment | Organism |
---|---|---|
alpha-Gal A | - |
Homo sapiens |
alpha-galactosidase A | - |
Homo sapiens |
Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
retains more than half of its activity after pre-incubation at pH 7.5 and 37°C for 90 min | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
4 | 4.5 | - |
Homo sapiens |
pH Minimum | pH Maximum | Comment | Organism |
---|---|---|---|
3 | 7.5 | wild type enzyme | Homo sapiens |
3.5 | 6.5 | mutant enzymes | Homo sapiens |
pH Stability | pH Stability Maximum | Comment | Organism |
---|---|---|---|
7.5 | - |
retains more than half of its activity after pre-incubation at pH 7.5 and 37°C for 90 min | Homo sapiens |