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Literature summary extracted from

  • Bijvoet, A.G.A.; Kroos, M.A.; Pieper, F.R.; van der Vliet, M.; de Boer, H.; van der Ploeg, A.T.; Verbeet, M.P.; Reuser, A.J.J.
    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.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.20 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)

EC Number Cloned (Comment) Organism
3.2.1.20 expression in mammary gland of transgenic mice Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.20 76000
-
mature recombinant enzyme, gel filtration Homo sapiens
3.2.1.20 110000
-
recombinant enzyme, precursor Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.20 additional information Homo sapiens glycogen storage disease is caused by lysosomal acid alpha-glucosidase deficiency ?
-
?

Organism

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

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.20 4-methylumbelliferyl-alpha-glucoside + H2O
-
Homo sapiens 4-methylumbelliferone + alpha-D-glucose
-
?
3.2.1.20 glycogen + H2O
-
Homo sapiens alpha-D-glucose
-
?
3.2.1.20 additional information glycogen storage disease is caused by lysosomal acid alpha-glucosidase deficiency Homo sapiens ?
-
?

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.2.1.20 4 4.5 hydrolysis of glycogen Homo sapiens
3.2.1.20 4.5 5 hydrolysis of 4-methylumbelliferyl alpha-glucoside Homo sapiens