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Literature summary for 3.2.1.46 extracted from
- Isoelectric focusing of galactosylceramide beta-galactosylceramide beta-galactosidase in cultured skin fibroblasts of patients with Krabbes globoid-cell leucodystrophy (1975), Biochem. Soc. Trans., 3, 241-244.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | enzyme deficiency in globoid cell leucodystrophy or Krabbe disease | 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 | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| skin fibroblast | in culture | 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 | - |
? | |
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