Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | in mucolipidosis patients | Homo sapiens | - |
- |
lysosome | in healthy humans | Homo sapiens | 5764 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
26000 | - |
1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE | Homo sapiens |
29000 | - |
1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme | Homo sapiens |
29500 | - |
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme | Homo sapiens |
54000 | - |
1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE | Homo sapiens |
55000 | - |
1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme | Homo sapiens |
55600 | - |
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme | Homo sapiens |
58000 | - |
1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme | Homo sapiens |
59600 | - |
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme | Homo sapiens |
68000 | - |
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
N-acetyl-beta-D-hexosaminides + H2O | Homo sapiens | - |
N-acetyl-D-hexosamines | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
healthy persons and mucolipidosis patients | - |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | mucolipidosis enzyme shows decreased carbohydrate composition compared to normal enzyme | Homo sapiens |
glycoprotein | carbohydrate modification | Homo sapiens |
glycoprotein | increased amount of galactose present on mucolipidosis enzyme compared to normal enzyme | Homo sapiens |
glycoprotein | galactosylation | Homo sapiens |
Purification (Comment) | Organism |
---|---|
to homogeneity, normal enzyme 64863fold, I-cell enzyme 20140fold | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
placenta | - |
Homo sapiens | - |
urine | - |
Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
77.84 | - |
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, normal enzyme | Homo sapiens |
90.63 | - |
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, I-cell enzyme | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside + H2O | - |
Homo sapiens | 4-methylumbelliferone + 2-acetamido-2-deoxy-beta-D-glucopyranoside | - |
? | |
N-acetyl-beta-D-hexosaminides + H2O | - |
Homo sapiens | N-acetyl-D-hexosamines | - |
? |
Subunits | Comment | Organism |
---|---|---|
dimer | 1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE | Homo sapiens |
tetramer | 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme | Homo sapiens |
trimer | 1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme | Homo sapiens |