Glycogen Debranching Enzyme System
"Glycogen Debranching Enzyme System" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D006004
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| MeSH Number(s) |
D08.811.277.450.420.450 D08.811.913.400.450.460.350
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| Concept/Terms |
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Below are MeSH descriptors whose meaning is more general than "Glycogen Debranching Enzyme System".
Below are MeSH descriptors whose meaning is more specific than "Glycogen Debranching Enzyme System".
This graph shows the total number of publications written about "Glycogen Debranching Enzyme System" by people in this website by year, and whether "Glycogen Debranching Enzyme System" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 1996 | 1 | 1 | 2 |
| 1997 | 3 | 0 | 3 |
| 1998 | 1 | 0 | 1 |
| 1999 | 1 | 0 | 1 |
| 2000 | 1 | 0 | 1 |
| 2002 | 1 | 0 | 1 |
| 2011 | 1 | 0 | 1 |
| 2015 | 1 | 0 | 1 |
| 2024 | 0 | 1 | 1 |
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Below are the most recent publications written about "Glycogen Debranching Enzyme System" by people in Profiles.
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Natural history study of hepatic glycogen storage disease type IV and comparison to Gbe1ys/ys model. JCI Insight. 2024 May 14; 9(12).
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A novel mouse model that recapitulates adult-onset glycogenosis type 4. Hum Mol Genet. 2015 Dec 01; 24(23):6801-10.
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The Saccharomyces cerevisiae fermentation stress response protein Igd1p/Yfr017p regulates glycogen levels by inhibiting the glycogen debranching enzyme. FEMS Yeast Res. 2011 Sep; 11(6):499-508.
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Molecular characterization of glycogen storage disease type III. Curr Mol Med. 2002 Mar; 2(2):167-75.
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Genotype-phenotype correlation in two frequent mutations and mutation update in type III glycogen storage disease. Mol Genet Metab. 2000 Jan; 69(1):16-23.
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Novel donor splice site mutations of AGL gene in glycogen storage disease type IIIa. J Inherit Metab Dis. 1999 Aug; 22(6):762-3.
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Two new mutations in the 3' coding region of the glycogen debranching enzyme in a glycogen storage disease type IIIa Ashkenazi Jewish patient. J Inherit Metab Dis. 1998 Apr; 21(2):141-8.
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A single-base deletion in the 3'-coding region of glycogen-debranching enzyme is prevalent in glycogen storage disease type IIIA in a population of North African Jewish patients. Eur J Hum Genet. 1997 Sep-Oct; 5(5):266-70.
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Polymorphic markers of the glycogen debranching enzyme gene allowing linkage analysis in families with glycogen storage disease type III. J Med Genet. 1997 Jan; 34(1):34-8.
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A nonsense mutation due to a single base insertion in the 3'-coding region of glycogen debranching enzyme gene associated with a severe phenotype in a patient with glycogen storage disease type IIIa. Hum Mutat. 1997; 9(1):37-40.