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Literature summary for 3.2.1.20 extracted from
- Isolation and characterisation of a recombinant, precursor form of lysosomal acid alpha-glucosidase (1995), Eur. J. Biochem., 234, 903-909.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | recombinant enzyme is taken up by cultured fibroblasts and skeletal muscle cells from glycogenosis type II patients, and is shown to correct the storage phenotype. Endocytosed enzyme is localised to the lysosome and shows evidence of intracellular processing to a more mature form | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| expression in Chinese hamster ovary K1 cells | Homo sapiens |
KM Value [mM]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 2.1 | - |
4-methylumbelliferyl-alpha-glucoside | recombinant enzyme | Homo sapiens |  |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 110000 | - |
x * 110000, recombinant enzyme, SDS-PAGE | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Homo sapiens | pompe disease, glycosidosis type II, is an autosomal recessive lysosomal storage disease that results from a deficiency of acid alpha-glucosidase. Patients with this disorder are unable to break down lysosomal glycogen, which consequently accumulates in the lysosome | ? | - |
? | |
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 | - |
? | |
| additional information | pompe disease, glycosidosis type II, is an autosomal recessive lysosomal storage disease that results from a deficiency of acid alpha-glucosidase. Patients with this disorder are unable to break down lysosomal glycogen, which consequently accumulates in the lysosome | Homo sapiens | ? | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 110000, recombinant enzyme, SDS-PAGE | Homo sapiens |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 4 | - |
hydrolysis of glycogen or maltose, recombinant enzyme | Homo sapiens |
| 4 | 5.5 | 4-methylumbelliferyl-alpha-glucoside | Homo sapiens |
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