Application | Comment | Organism |
---|---|---|
medicine | enzyme deficiency in globoid cell leucodystrophy or Krabbe disease | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
121000 | - |
gel filtration, monomer | Homo sapiens |
125000 | - |
1 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |
125000 | - |
2 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |
125000 | - |
4 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |
125000 | - |
6 * 125000, active form in vivo, SDS-PAGE | Homo sapiens |
760000 | - |
gel filtration, hexamer | 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 | - |
normal enzyme | - |
Homo sapiens | - |
mutant enzyme /Krabbe disease | - |
Purification (Comment) | Organism |
---|---|
to homogeneity, Krabbe disease | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
liver | - |
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 | - |
? |
Subunits | Comment | Organism |
---|---|---|
dimer | 2 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |
hexamer | 6 * 125000, active form in vivo, SDS-PAGE | Homo sapiens |
monomer | 1 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |
tetramer | 4 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE | Homo sapiens |