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Literature summary extracted from

  • Prince, W.S.; McCormick, L.M.; Wendt, D.J.; Fitzpatrick, P.A.; Schwartz, K.L.; Aguilera, A.I.; Koppaka, V.; Christianson, T.M.; Vellard, M.C.; Pavloff, N.; Lemontt, J.F.; Qin, M.; Starr, C.M.; Bu, G.; Zankel, T.C.
    Lipoprotein receptor binding, cellular uptake, and lysosomal delivery of fusions between the receptor-associated protein (RAP) and alpha-L-iduronidase or acid alpha-glucosidase (2004), J. Biol. Chem., 279, 35037-35046.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.76 medicine the enzyme is a target for enzyme replacement therapy of lysosomal storage disorders of mucopolysaccharidosis type I patients, the therapy depends on efficient uptake of recombinant enzyme into tissues of patients Homo sapiens
3.2.1.76 pharmacology exploitation of alternative receptor systems that are independent of glycosylation but allow for efficient delivery to the lysosome Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.2.1.76 expression of wild-type enzyme and enzyme in fusion with the human receptor-associated protein, RAP, in LRP1-null CHO-K1 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.2.1.76 additional information exploitation of alternative receptor systems that are independent of glycosylation but allow for efficient delivery to the lysosome, fusions of the human lysosomal enzyme with the receptor-associated protein, RAP, are efficiently endocytosed by lysosomal storage disorder patient fibroblasts, rat C6 glioma cells, mouse C2C12 myoblasts, and recombinant CHO cells expressing individual members of the low-density lipoprotein receptor family, uptake of the fusion exceeds that of phosphorylated enzyme in all cases, uptake is mediated by oligosaccharide receptors including the cation-independent mannose 6-phosphate receptor and the mannose receptor, and is specifically mediated by members of the low-density lipoprotein receptor protein family and is followed by delivery of the fusions to the lysosome, stability or recombinant fusion enzymes, overview Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.2.1.76 0.3
-
 4-methylumbelliferyl alpha-L-iduronide pH 5.8, 37°C, recombinant enzyme Homo sapiens

Localization

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

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.76 83000
-
 x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE Homo sapiens
3.2.1.76 125000
-
 x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.76 Homo sapiens
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.2.1.76 glycoprotein oligomannose 7-bisphosphate is bound to the enzyme, with a much higher amount on the recombinant wild-type enzyme compared to the recombinant RAP fusion enzyme Homo sapiens

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.76 recombinant wild-type enzyme and human receptor-associated protein RAP-fusion enzyme from LRP1-null CHO-K1 cells by heparin affinity and hydrophobic interaction chromatography, and gel fitration Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.76 fibroblast
-
 Homo sapiens
-
3.2.1.76 liver
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.76 4-methylumbelliferyl-alpha-L-iduronide + H2O
-
 Homo sapiens 4-methylumbelliferone + alpha-L-iduronic acid
-
 ?

Subunits

EC Number Subunits Comment Organism
3.2.1.76 ? x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.2.1.76 alpha-L-iduronidase
-
 Homo sapiens
3.2.1.76 IDU
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.2.1.76 37
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.2.1.76 5.8
-
 assay at Homo sapiens