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DeCS
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Descriptor English:
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Glycogen Debranching Enzyme System
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Descriptor Spanish:
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Sistema de la Enzima Desramificadora del Glucógeno
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Descriptor Portuguese:
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Sistema da Enzima Desramificadora do Glicogênio
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Synonyms English:
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Debranching Enzyme, Glycogen
Enzyme, Glycogen Debranching
Glycogen Debranching Enzyme
Transfer Glucosidase
Transfer-Glucosidase
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Tree Number:
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D08.811.277.450.420.450
D08.811.913.400.450.460.350
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Definition English:
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1,4-alpha-D-Glucan-1,4-alpha-D-glucan 4-alpha-D-glucosyltransferase/dextrin 6 alpha-D-glucanohydrolase. An enzyme system having both 4-alpha-glucanotransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) activities. As a transferase it transfers a segment of a 1,4-alpha-D-glucan to a new 4-position in an acceptor, which may be glucose or another 1,4-alpha-D-glucan. As a glucosidase it catalyzes the endohydrolysis of 1,6-alpha-D-glucoside linkages at points of branching in chains of 1,4-linked alpha-D-glucose residues. Amylo-1,6-glucosidase activity is deficient in glycogen storage disease type III. |
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Indexing Annotation English:
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/defic: consider also GLYCOGEN STORAGE DISEASE TYPE III
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See Related English:
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Glycogen Storage Disease Type III
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History Note English:
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89(76); was GLYCOGEN DEBRANCHING ENZYME see under GLUCOSYLTRANSFERASES 1976-88
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Allowable Qualifiers English:
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Record Number:
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24391
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Unique Identifier:
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D006004
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Occurrence in VHL:
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Similar:
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DeCS
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