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Literature summary for 3.2.1.33 extracted from

  • Justice, P.; Ryan, C.; Hsia, D.Y.Y.; Krmpotik, E.
    Amylo-1,6-glucosidase in human fibroblasts: studies in type III glycogen storage disease (1970), Biochem. Biophys. Res. Commun., 39, 301-306.
    View publication on PubMed

Application

Application Comment Organism
medicine diagnosis, even prenatal, of type III glycogen storage disease: marked decrease or absence of amylo-1,6-glucosidase, glycogen debranching enzyme Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
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patients with type I and type III glycogen storage disease and control patient
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Source Tissue

Source Tissue Comment Organism Textmining
amniotic fluid cultivated Homo sapiens
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fibroblast from skin biopsies Homo sapiens
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leukocyte
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Homo sapiens
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liver
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Homo sapiens
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muscle
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Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
glycogen + H2O reverse reaction: incorporation of glucose into glycogen Homo sapiens glycogen + D-glucose
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r

Synonyms

Synonyms Comment Organism
glycogen debranching system EC 3.2.1.33 found in mammals and yeast is in a single polypeptide chain containing two active centres. The other activity is similar to that of EC 2.4.1.25, 4-alpha-glucanotransferase, which acts on the glycogen phosphorylase limit dextrin chains to expose the single glucose residues, which the 6-alpha-glucosidase activity can hydrolyse. Together, these two activities constitute the glycogen debranching system Homo sapiens