Protein Variants | Comment | Organism |
---|---|---|
K104A | mutation does not result in any altered activity | Homo sapiens |
K105A | intracellular activity is not reduced but zinc-responsive secreted activity is reduced | Homo sapiens |
K118A | intracellular activity is not reduced but zinc-responsive secreted activity is reduced | Homo sapiens |
K124A | intracellular activity is not reduced but zinc-responsive secreted activity is reduced | Homo sapiens |
K183A | intracellular activity is not reduced but zinc-responsive secreted activity is reduced | Homo sapiens |
K187A | intracellular activity is reduced but zinc-responsive secreted activity is unchanged | Homo sapiens |
K249A | intracellular activity and zinc-responsive secreted activity are both reduced | Homo sapiens |
K305A | intracellular activity and zinc-responsive secreted activity are both reduced | Homo sapiens |
K346A | intracellular activity is reduced but zinc-responsive secreted activity is unchanged | Homo sapiens |
K419A | intracellular activity and zinc-responsive secreted activity are both reduced | Homo sapiens |
K433A | intracellular activity and zinc-responsive secreted activity are both reduced | Homo sapiens |
K576A | intracellular activity and zinc-responsive secreted activity are both reduced | Homo sapiens |
K93A | mutant enzyme has reduced intracellular activity, but enhanced zinc-responsive secreted activity | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
intracellular | the ratio of secreted to intracellular activity is about 8fold greater in I-cells (skin fibroblasts from I-cell disease patients, in which one of the enzymes responsible for mannose phosphorylation, GlcNAc-phosphotransferase, is deficient) than in normal cells | Homo sapiens | 5622 | - |
lysosome | can rapidly move from lysosomes to cell surface upon stimulation by cytokines and other factors | Homo sapiens | 5764 | - |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Zn2+ | most of the secreted activity requires Zn2+ for full activity | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | an inherited deficiency of this enzymatic activity results in the type A and B forms of Niemann-Pick disease | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
phosphoprotein | mannose phosphorylation is important in the trafficking of this enzyme | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | an inherited deficiency of this enzymatic activity results in the type A and B forms of Niemann-Pick disease | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
acid spingomyelinase | - |
Homo sapiens |