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Literature summary for 3.2.1.76 extracted from
- 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.
Application
| Application | Comment | Organism |
|---|---|---|
| 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 |
| pharmacology | exploitation of alternative receptor systems that are independent of glycosylation but allow for efficient delivery to the lysosome | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| 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
| Protein Variants | Comment | Organism |
|---|---|---|
| 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]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.3 | - |
4-methylumbelliferyl alpha-L-iduronide | pH 5.8, 37°C, recombinant enzyme | 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 |
|---|---|---|---|
| 83000 | - |
x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE | Homo sapiens |
| 125000 | - |
x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE | Homo sapiens |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| 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)
| Purification (Comment) | Organism |
|---|---|
| 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
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| fibroblast | - |
Homo sapiens | - |
| liver | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 4-methylumbelliferyl-alpha-L-iduronide + H2O | - |
Homo sapiens | 4-methylumbelliferone + alpha-L-iduronic acid | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 83000, recombinant wild-type enzyme, SDS-PAGE, x * 125000, recombinant RAP-fusion enzyme, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| alpha-L-iduronidase | - |
Homo sapiens |
| IDU | - |
Homo sapiens |
Temperature Optimum [°C]
| Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|
| 37 | - |
assay at | Homo sapiens |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 5.8 | - |
assay at | Homo sapiens |
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