Alpha-1,4-transglycosylation Activity of GH57 Glycogen Branching Enzymes Is Higher in the Absence of a Flexible Loop with a Conserved Tyrosine Residue [PDF]
, 2023 Starch-like polymers can be created through the use of enzymatic modification with glycogen branching enzymes (GBEs). GBEs are categorized in the glycoside hydrolase (GH) family 13 and 57. Both GH13 and GH57 GBEs exhibit branching and hydrolytic activity.
Bax, Hilda Hubertha Maria +2 more
core +1 more source
Stem Cell Research, 2023 Glycogen storage disease type III (GSDIII) is an autosomal recessive disorder characterized by a deficiency of glycogen debranching enzyme (GDE) leading to cytosolic glycogen accumulation and inducing liver and muscle pathology.
Lucille Rossiaud +5 more
doaj
Biomédica: revista del Instituto Nacional de Salud, 2018 Introduction: Type III glycogen storage disease (GSD III) is an autosomal recessive disorder in which a mutation in the AGL gene causes deficiency of the glycogen debranching enzyme.
Carolina Mantilla +8 more
doaj +1 more source
Liver transplantation for type I and type IV glycogen storage disease [PDF]
, 1993 Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period.
A. G. Tzakis +37 more
core +1 more source
Activity of porcine muscle glycogen debranching enzyme in relation to pH and temperature [PDF]
Meat Science, 2005 The activity of glycogen debranching enzyme (GDE) was studied in relation to pH value and temperature in porcine masseter and longissimus dorsi muscles. A glycogen limit dextrin was used as the substrate for GDE, and the enzyme was derived from raw meat extracts. In both muscles, the pH only weakly affected the activity of GDE at the pH values found in
Marita Ruusunen +2 more
openaire +3 more sources
MIRAS phasing of the crystal structure of the glycogen debranching enzyme from mammalian muscle [PDF]
Acta Crystallographica Section A Foundations of Crystallography, 1996 J. P. W. Petersen +3 more
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Phylogenetic study of glycogen synthesizing enzymes in Synechocystis [PDF]
, 2004 Glycogen is a major reserve carbohydrate in both prokaryotes and eukaryotes, and starch is a major reserve carbohydrate only in photosynthetic eukaryotes.
He, Wei
core +3 more sources
Molecular Plant-Microbe Interactions, 2002 The phosphoglucomutase (pgm) gene codes for a key enzyme required for the formation of UDP-glucose and ADP-glucose, the sugar donors for the biosynthesis of glucose containing polysaccharides.
Viviana C. Lepek +5 more
doaj +1 more source
The Management and Clinical Outcomes of Pregnancy in a Female With Glycogen Storage Disease Type IIIA Caused by Rare Variant [PDF]
JIMD RepABSTRACT Glycogen storage disease type III (GSD III) is an inborn error of carbohydrate metabolism with an autosomal recessive inheritance pattern. Phenotypically, patients can manifest a broad clinical spectrum. Most patients affected with GSD IIIA (85%) have a non‐functional GDE enzyme primarily affecting the liver and cardiac/skeletal muscle (Type ...
Puente‐Ruiz N +9 more
europepmc +2 more sources
Functional and structural characterization of plastidic starch phosphorylase during barley endosperm development [PDF]
, 2017 The production of starch is essential for human nutrition and represents a major metabolic flux in the biosphere. The biosynthesis of starch in storage organs like barley endosperm operates via two main pathways using different substrates: starch ...
Cuesta-Seijo, Jose A. +6 more
core +1 more source