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Literature summary for 3.2.1.20 extracted from
- Recombinant human acid alpha-glucosidase: high level production in mouse milk, biochemical characteristics, correction of enzyme deficiency in GSDII KO mice (1998), Hum. Mol. Genet., 7, 1815-1824.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | glycogen storage disease is caused by lysosomal acid alpha-glucosidase deficiency. When injected intravenously, the recombinant enzyme from mouse milk corrects the acid alpha-glucosidase deficiency in heart and skeletal muscle of glycogen storage disease type II knockout mice | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| expression in mammary gland of transgenic mice | Homo sapiens |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 76000 | - |
mature recombinant enzyme, gel filtration | Homo sapiens |
| 110000 | - |
recombinant enzyme, precursor | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Homo sapiens | glycogen storage disease is caused by lysosomal acid alpha-glucosidase deficiency | ? | - |
? |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 4-methylumbelliferyl-alpha-glucoside + H2O | - |
Homo sapiens | 4-methylumbelliferone + alpha-D-glucose | - |
? | |
| glycogen + H2O | - |
Homo sapiens | alpha-D-glucose | - |
? | |
| additional information | glycogen storage disease is caused by lysosomal acid alpha-glucosidase deficiency | Homo sapiens | ? | - |
? | |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 4 | 4.5 | hydrolysis of glycogen | Homo sapiens |
| 4.5 | 5 | hydrolysis of 4-methylumbelliferyl alpha-glucoside | Homo sapiens |
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Release 2023.1 (January 2023)
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