Application | Comment | Organism |
---|---|---|
medicine | enzyme deficiency in globoid cell leucodystrophy or Krabbe disease | Homo sapiens |
General Stability | Organism |
---|---|
urea, 50% loss of activity of the mutant enzyme at 1.3 M urea, 50% loss of activity of the normal enzyme at 5.6 M urea | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.02 | - |
galactosylceramide | normal enzyme | Homo sapiens | |
0.165 | - |
galactosylceramide | mutant enzyme | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
750000 | - |
gel filtration, SDS-PAGE, mutant and normal protein | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
D-galactosyl-N-acylsphingosine + H2O | Homo sapiens | - |
? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
mutant enzyme /Krabbe disease | - |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
liver | - |
Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
0.12 | - |
- |
Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl beta-galactoside + H2O | - |
Homo sapiens | methylumbelliferone + beta-D-galactose | - |
? | |
D-galactosyl-N-acylsphingosine + H2O | - |
Homo sapiens | D-galactose + N-acylsphingosine | - |
? | |
D-galactosyl-N-acylsphingosine + H2O | - |
Homo sapiens | ? | - |
? | |
galactocerebroside + H2O | - |
Homo sapiens | D-galactose + N-acylceramide | - |
? | |
galactosylceramide + H2O | - |
Homo sapiens | D-galactose + N-acylceramide | - |
? | |
lactosylsphingosine + H2O | - |
Homo sapiens | lactose + sphingosine | - |
? |
Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
---|---|---|---|
52 | - |
4 min, 50% loss of activity, mutant enzyme | Homo sapiens |
52 | - |
35 min, 50% loss of activity, normal enzyme | Homo sapiens |