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Literature summary for 3.2.1.46 extracted from
- Characterization of 6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside as fluorogenic substrate of galactocerebrosidase for the diagnosis of Krabbe disease (1992), Clin. Chim. Acta, 205, 87-96.
Activating Compound
| Activating Compound | Comment | Organism | Structure |
|---|---|---|---|
| taurocholate | in combination with oleic acid | Mus musculus |  |
| taurocholate | in combination with oleic acid | Homo sapiens | |
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | enzymatic diagnosis | Mus musculus |
| medicine | enzymatic diagnosis | Homo sapiens |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| 6-hexadecanoylamino-4-methylbelliferyl-beta-D-galactopyranoside | competitive | Homo sapiens | |
| 6-hexadecanoylamino-4-methylbelliferyl-beta-D-galactopyranoside | competitive | Mus musculus | |
KM Value [mM]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.03 | - |
6-hexadecanoylamino-4-methylbelliferyl-beta-D-galactopyranoside | taurocholate and oleic acid activated, in leukocytes | Homo sapiens | |
| 0.04 | - |
6-hexadecanoylamino-4-methylbelliferyl-beta-D-galactopyranoside | taurocholate and oleic acid activated, in fibroblasts | Homo sapiens | |
| 0.15 | - |
6-hexadecanoylamino-4-methylbelliferyl-beta-D-galactopyranoside | taurocholate and oleic acid activated | Mus musculus | |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| D-galactosyl-N-acylsphingosine + H2O | Mus musculus | - |
? | - |
? | |
| D-galactosyl-N-acylsphingosine + H2O | Homo sapiens | - |
? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
| Mus musculus | - |
twitcher mouse | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| brain | - |
Homo sapiens | - |
| kidney | - |
Mus musculus | - |
| leukocyte | - |
Homo sapiens | - |
| skin fibroblast | in culture | Mus musculus | - |
| skin fibroblast | in culture | Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside + H2O | fluorogenic substrate | Mus musculus | 6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose | - |
? | |
| 6-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranoside + H2O | fluorogenic substrate | Homo sapiens | 6-hexadecanoylamino-4-methylumbelliferone + beta-D-galactopyranose | - |
? | |
| D-galactosyl-N-acylsphingosine + H2O | - |
Mus musculus | D-galactose + N-acylsphingosine | - |
? | |
| D-galactosyl-N-acylsphingosine + H2O | - |
Homo sapiens | D-galactose + N-acylsphingosine | - |
? | |
| D-galactosyl-N-acylsphingosine + H2O | - |
Mus musculus | ? | - |
? | |
| D-galactosyl-N-acylsphingosine + H2O | - |
Homo sapiens | ? | - |
? | |
| galactocerebroside + H2O | - |
Mus musculus | D-galactose + N-acylceramide | - |
? | |
| galactocerebroside + H2O | - |
Homo sapiens | D-galactose + N-acylceramide | - |
? | |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 4.5 | - |
taurocholate and oleic acid activated | Mus musculus |
| 4.5 | - |
taurocholate and oleic acid activated | Homo sapiens |
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