Preferred Label : glycogen debranching enzyme system;
MeSH definition : 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.;
MeSH synonym : transfer glucosidase; transfer-glucosidase;
MeSH hyponym : glycogen debranching enzyme; Debranching Enzyme, Glycogen; Enzyme, Glycogen Debranching;
MeSH annotation : /defic: consider also GLYCOGEN STORAGE DISEASE TYPE III;
Wikipedia link : https://en.wikipedia.org/wiki/Glycogen debranching enzyme;
Is substance : O;
Origin ID : D006004;
UMLS CUI : C0017915;
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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.
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=366
2009
true
France
French
glycogen debranching enzyme system
glycogen storage disease type iii
scientific and technical information
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