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Literature summary for 3.2.1.46 extracted from
- Galactosylceramide beta-galactosidase in Krabbe disease: partial purification and characterization of the mutant enzyme (1979), Arch. Biochem. Biophys., 196, 93-101.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | enzyme deficiency in globoid cell leucodystrophy or Krabbe disease | Homo sapiens |
General Stability
| 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 [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 [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 750000 | - |
gel filtration, SDS-PAGE, mutant and normal protein | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| D-galactosyl-N-acylsphingosine + H2O | Homo sapiens | - |
? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
mutant enzyme /Krabbe disease | - |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
- |
Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| liver | - |
Homo sapiens | - |
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| 0.12 | - |
- |
Homo sapiens |
Substrates and Products (Substrate)
| 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 [°C]
| 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 |
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