EC Number | Cloned (Comment) | Organism |
---|---|---|
3.1.6.12 | expression of ASB C53S mutant in CHO cells | Homo sapiens |
EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
3.1.6.12 | C53S | the mutation causes ASB-deficiency, phenoytpe, overview. The enzyme is taken up into cultured ASB-deficient human fibroblasts, GM00519 cells, and translocates to the lysosomes, it is catalytically active. The enzyme enters target cells predominantly through the CI-M6P receptor. The uptake of rhASB is able to restore lysosomal function in an in vitro cell-based assay | Homo sapiens |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
3.1.6.12 | lysosome | - |
Homo sapiens | 5764 | - |
EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
3.1.6.12 | chondroitin sulfate + H2O | Homo sapiens | - |
? | - |
? | |
3.1.6.12 | dermatan sulfate + H2O | Homo sapiens | - |
? | - |
? | |
3.1.6.12 | additional information | Homo sapiens | ASB is a lysosomal exohydrolase, cleaving the 4-sulfate from the N-acetylgalactosamine-4-sulfate residue at the nonreducing terminal of glycosaminoglycan structures | ? | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
3.1.6.12 | Homo sapiens | - |
- |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
3.1.6.12 | fibroblast | - |
Homo sapiens | - |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
3.1.6.12 | chondroitin sulfate + H2O | - |
Homo sapiens | ? | - |
? | |
3.1.6.12 | dermatan sulfate + H2O | - |
Homo sapiens | ? | - |
? | |
3.1.6.12 | additional information | ASB is a lysosomal exohydrolase, cleaving the 4-sulfate from the N-acetylgalactosamine-4-sulfate residue at the nonreducing terminal of glycosaminoglycan structures | Homo sapiens | ? | - |
? |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
3.1.6.12 | arylsulfatase B | - |
Homo sapiens |
3.1.6.12 | ASB | - |
Homo sapiens |
EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|---|
3.1.6.12 | 37 | - |
assay at | Homo sapiens |
EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|---|
3.1.6.12 | 8.2 | - |
assay at | Homo sapiens |
EC Number | General Information | Comment | Organism |
---|---|---|---|
3.1.6.12 | malfunction | ASB-deficient cells accumulate dermatan sulfate and chondroitin sulfate, which may be partially hydrolyzed by other lysosomal hydrolases | Homo sapiens |