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Literature summary for 3.1.6.13 extracted from

  • Villani, G.R.; Daniele, A.; Balzano, N.; Di Natale, P.
    Expression of five iduronate-2-sulfatase site-directed mutations (2000), Biochim. Biophys. Acta, 1501, 71-80.
    View publication on PubMed
Search for more literature for 3.1.6.13

Application

Application Comment Organism
medicine the inherited deficiency results in mucopolysaccharidosis type II, the Hunter syndrome Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression in COS 7 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information R88H, R88P, T118I, 959delT, R468Q mutations are identified in Hunter syndrome patients Homo sapiens
R88H 13.7% residual enzyme activity in comparison to wild-type enzyme Homo sapiens
R88P total absence of residual activity Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.133
-
 O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate mutant R88H Homo sapiens
0.327
-
 O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate wild-type enzyme Homo sapiens

Localization

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

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the enzyme is responsible for heparin sulfate and dermatan sulfate degradation ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information the enzyme is responsible for heparin sulfate and dermatan sulfate degradation Homo sapiens ?
-
 ?
O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate
-
 Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
? COS 7 cells transfected with wild-type enzyme expression vector, produce three polypeptides of 90000, 62000 and 45000 Da, corresponding to precursor, intermediate and mature forms of enzyme, pulse-chase labelling Homo sapiens