Results 1 to 10 of about 2,890 (187)

Molecular architecture and catalytic mechanism of human glycogen debranching enzyme [PDF]

Nature Communications
Glycogen, a key branched glucose polymer, acts as a vital energy reservoir in mammalian cells, particularly during intense activity or fasting. The glycogen debranching enzyme (GDE) plays a key role in glycogen degradation by removing branches, ensuring ...
Huiyi Guan, Huan Chen, He Geng, Ruifang Ma, Zhongmin Liu, Yong Wang, Yifang Chen, Kaige Yan   +7 more
doaj   +5 more sources

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, Pollard C, Ru Y, Ritterson Lew C, Duex JE, Dancik G, Owens C, Spencer A, Knight S, Holemon H, Gupta S, Hansel D, Hellerstein M, Lorkiewicz P, Lane AN, Fan TW, Theodorescu D.   +16 more
europepmc   +5 more sources

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, Lingling Feng, Lin Xia, Huiyong Yin, Song Xiang   +4 more
doaj   +4 more sources

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, Su Jin Choi, Fengqin Gao, Priya S. Kishnani, Baodong Sun   +4 more
doaj   +4 more sources

Involvement of glycogen debranching enzyme in bladder cancer. [PDF]

Biomed Rep, 2017
Bladder cancer is the most common malignancy of the urinary system, however the molecular pathways underlying this disease are incompletely understood. To understand new regulators of bladder cancer progression, the authors carried out a functional genomic screen which identified glycogen debranching enzyme (AGL) as a novel regulator of bladder cancer ...
Weinhaus B, Guin S.
europepmc   +6 more sources

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   +5 more sources

Physical exercise intervention in glycogen storage disease IIIa: Feasibility and multisystem benefits [PDF]

Experimental Physiology
Glycogen storage disease III (GSD‐III) is caused by an inherited deficiency of the glycogen debranching enzyme. Affecting the liver, muscle and heart, GSD‐IIIa is the most common GSD‐III subtype.
Asunción Bustos‐Sellers, Almudena Montalvo‐Pérez, Araceli Boraita, Gonzalo Saco‐Ledo, Lidia B. Alejo, David Barranco‐Gil, Pedro L. Valenzuela, Nerea López‐Maldonado, Roser Ferrer‐Costa, Javier Esparcia‐Estrela, Celia Monteagudo‐López, Tomàs Pinós, Alejando Santos‐Lozano, Alejandro Lucia, Alfredo Santalla   +14 more
doaj   +3 more sources

Identification of the Catalytic Residues of Bifunctional Glycogen Debranching Enzyme [PDF]

Journal of Biological Chemistry, 2001
Eukaryotic glycogen debranching enzyme (GDE) possesses two different catalytic activities (oligo-1,4-->1,4-glucantransferase/amylo-1,6-glucosidase) on a single polypeptide chain. To elucidate the structure-function relationship of GDE, the catalytic residues of yeast GDE were determined by site-directed mutagenesis. Asp-535, Glu-564, and Asp-670 on the
Akifumi Nakayama, Keizô Yamamoto, Shiro Tabata   +2 more
openalex   +4 more sources

Mono-(2-ethylhexyl) phthalate Targets Glycogen Debranching Enzyme and Affects Glycogen Metabolism in Rat Testis [PDF]

Toxicological Sciences, 2009
Phthalate esters are commonly used plasticizers; however, some are suspected to cause reproductive toxicity. Administration of high doses of di-(2-ethylhexyl) phthalate (DEHP) induces germ cell death in male rodents. Mono-(2-ethylhexyl) phthalate (MEHP), a hydrolyzed metabolite of DEHP, appears to be responsible for this testicular toxicity; however ...
Chikanori Kuramori, Yasuyoshi Hase, Koichi Hoshikawa, Keiko Watanabe, Takeyuki Nishi, Takako Hishiki, Tomoyoshi Soga, Akihiro Nashimoto, Yasuaki Kabe, Yuki Yamaguchi, Hajime Watanabe, Kohsuke Kataoka, Makoto Suematsu, Hiroshi Handa   +13 more
openalex   +3 more sources

Glycogen Storage Disease in Twins: When Two Lives Reflect One Silent Battle. [PDF]

Clin Case Rep
ABSTRACT Early recognition of hepatomegaly, hypoglycemia, and elevated liver enzymes in infants is crucial for diagnosing glycogen storage disease. Liver biopsy with PAS–diastase staining provides definitive confirmation. Prompt metabolic management and regular follow‐up are essential to prevent progression of fibrosis and long‐term hepatic ...
Shah RK, Khan SA, Devkota D, Aryal M, Subedi N, Aryal B, Gurubacharya B, Parajuli S, Gupta A, Parajuli SB, Kausar H.   +10 more
europepmc   +2 more sources