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Literature summary for 3.1.6.8 extracted from

  • Schroeder, S.; Matthes, F.; Hyden, P.; Andersson, C.; Fogh, J.; Mueller-Loennies, S.; Braulke, T.; Gieselmann, V.; Matzner, U.
    Site-specific analysis of N-linked oligosaccharides of recombinant lysosomal arylsulfatase A produced in different cell lines (2010), Glycobiology, 20, 248-259.
    View publication on PubMed
Search for more literature for 3.1.6.8

Application

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(Commentary)

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

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
 Homo sapiens 5764
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
62000
-
 x * 62000, SDS-PAGE Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

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

Subunits Comment Organism
? x * 62000, SDS-PAGE Homo sapiens

Synonyms

Synonyms Comment Organism
arylsulfatase A
-
 Homo sapiens
ASA
-
 Homo sapiens

General Information

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