Cloned (Comment) | Organism |
---|---|
expression of ASB C53S mutant in CHO cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
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 |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Homo sapiens | 5764 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
chondroitin sulfate + H2O | Homo sapiens | - |
? | - |
? | |
dermatan sulfate + H2O | Homo sapiens | - |
? | - |
? | |
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 | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
chondroitin sulfate + H2O | - |
Homo sapiens | ? | - |
? | |
dermatan sulfate + H2O | - |
Homo sapiens | ? | - |
? | |
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 | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
arylsulfatase B | - |
Homo sapiens |
ASB | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
8.2 | - |
assay at | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | ASB-deficient cells accumulate dermatan sulfate and chondroitin sulfate, which may be partially hydrolyzed by other lysosomal hydrolases | Homo sapiens |