Crystal structure of glycogen debranching enzyme and insights into its catalysis and disease-causing mutations [PDF]
Nature Communications, 2016 Debranching of glycogen is an important step in its use as an energy source. Here, the authors describe the crystal structures of glycogen debranching enzyme alone and in complex with oligosaccharides and provide molecular insights into the function, and
Liting Zhai +4 more
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A Novel Gene Therapy Approach for GSD III Using an AAV Vector Encoding a Bacterial Glycogen Debranching Enzyme [PDF]
Molecular Therapy: Methods & Clinical Development, 2020 Glycogen storage disease type III (GSD III) is an inherited disorder caused by a deficiency of glycogen debranching enzyme (GDE), which results in the accumulation of abnormal glycogen (limit dextrin) in the cytoplasm of liver, heart, and skeletal muscle
Jeong-A Lim +4 more
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Role in tumor growth of a glycogen debranching enzyme lost in glycogen storage disease. [PDF]
J Natl Cancer Inst, 2014 Bladder cancer is the most common malignancy of the urinary system, yet our molecular understanding of this disease is incomplete, hampering therapeutic advances.Here we used a genome-wide functional short-hairpin RNA (shRNA) screen to identify suppressors of in vivo bladder tumor xenograft growth (n = 50) using bladder cancer UMUC3 cells.
Guin S +16 more
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Crystal structures of glycogen-debranching enzyme mutants in complex with oligosaccharides. [PDF]
Acta Crystallogr F Struct Biol Commun, 2021 Debranching is a critical step in the mobilization of the important energy store glycogen. In eukaryotes, including fungi and animals, the highly conserved glycogen-debranching enzyme (GDE) debranches glycogen by a glucanotransferase (GT) reaction followed by a glucosidase (GC) reaction. Previous work indicated that these reactions are catalyzed by two
Shen M, Gong X, Xiang S.
europepmc +6 more sources
The Synergistic Effect of GH13 and GH57 GBEs of Petrotoga mobilis Results in α-Glucan Molecules with a Higher Branch Density. [PDF]
Polymers (Basel), 2023 Glycogen is a biopolymer consisting of glycosyl units, with a linear backbone connected by α-1,4-linkages and branches attached via α-1,6-linkages. In microorganisms, glycogen synthesis involves multiple enzymes, with glycogen branching enzymes (GBEs ...
Bax HHM +3 more
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The Autophagic Activator GHF-201 Can Alleviate Pathology in a Mouse Model and in Patient Fibroblasts of Type III Glycogenosis [PDF]
BiomoleculesGlycogen storage disease type III (GSDIII) is a hereditary glycogenosis caused by deficiency of the glycogen debranching enzyme (GDE), an enzyme, encoded by Agl, enabling glycogen degradation by catalyzing alpha-1,4-oligosaccharide side chain transfer ...
Kumudesh Mishra +11 more
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Mitochondrial Dysfunction in Glycogen Storage Disorders (GSDs) [PDF]
BiomoleculesGlycogen storage disorders (GSDs) are a group of inherited metabolic disorders characterized by defects in enzymes involved in glycogen metabolism. Deficiencies in enzymes responsible for glycogen breakdown and synthesis can impair mitochondrial function.
Kumudesh Mishra, Or Kakhlon
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Diverse electrophysiological demyelinating features in a late-onset glycogen storage disease type IIIa case [PDF]
Open MedicineGlycogen storage disease type IIIa (GSD IIIa) is a rare etiology among patients with adult-onset myopathy, which is typically associated with axonopathy rather than demyelination.
Zhou Xiajun +12 more
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Molecular architecture and catalytic mechanism of human glycogen debranching enzyme. [PDF]
Nat CommunGuan H +7 more
europepmc +2 more sources
CD44 and RHAMM are essential for rapid growth of bladder cancer driven by loss of Glycogen Debranching Enzyme (AGL). [PDF]
BMC Cancer, 2016 Oldenburg D +5 more
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