Application | Comment | Organism |
---|---|---|
medicine | enzyme replacement therapy is a therapeutic option for metachromatic leukodystrophy, caused by enzyme-deficiency, and other lysosomal disorders. This therapy depends on N-linked oligosaccharide-mediated delivery of intravenously injected recombinant enzyme to the lysosomes of patient cells | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
recombinant expression in CHO, BHK and HT-1080 cells. High variability of the high-mannose-type N-glycans, which prevail at all glycosylation sites, depending on the culture conditions and the cell line expressing the enzyme, overview | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Homo sapiens | 5764 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
62000 | - |
x * 62000, SDS-PAGE | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | three N-glycosylation sites and three N-linked oligosaccharide side chains at asparagine residues 158, 184, and 350, the recombinantly expressed enzyme shows a high variability of the high-mannose-type N-glycans, which prevail at all glycosylation sites, depending on the culture conditions and the cell line expressing the enzyme, overview. The composition of the glycans is largely determined by substantial trimming in the medium, the susceptibility for trimming is different for the glycans at the three N-glycosylation sites, which of the glycans is most susceptible to trimming also depends on production conditions. Structure and ligand binding analysis, overview | Homo sapiens |
Subunits | Comment | Organism |
---|---|---|
? | x * 62000, SDS-PAGE | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
arylsulfatase A | - |
Homo sapiens |
ASA | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | metachromatic leukodystrophy, MLD, is a lysosomal storage disease caused by a deficiency of the lysosomal enzyme arylsulfatase A | Homo sapiens |
metabolism | arylsulfatase A catalyzes the first step in the intralysosomal degradation of the sphingolipid 3-O-sulfogalactosylceramide, briefly called sulfatide | Homo sapiens |