Activating Compound | Comment | Organism | Structure |
---|---|---|---|
additional information | When islets are cultured in different concentrations of D-glucose, the levels of enzyme mRNA increases, in a dose-dependent manner. Mannoheptulose completely blocks the effect of glucose. | Mus musculus |
Application | Comment | Organism |
---|---|---|
medicine | in humans, the inherited deficiency of the enzyme activity results in mucopolysaccharidosis type II, the Hunter syndrome | Mus musculus |
Cloned (Comment) | Organism |
---|---|
expressed in islets, alpha and beta clonal cells | Mus musculus |
Protein Variants | Comment | Organism |
---|---|---|
additional information | in 25 Hunter syndrome patients 20 mutations of the gene are identified of which 13 mutations are novel | Mus musculus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Mus musculus | 5764 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Mus musculus | The enzyme has a potential role in normal pathway of lysosomal degradation of secretory peptides and is likely to be essential to maintain pancreatic beta-cell function | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
pancreatic islet | - |
Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | The enzyme has a potential role in normal pathway of lysosomal degradation of secretory peptides and is likely to be essential to maintain pancreatic beta-cell function | Mus musculus | ? | - |
? |