About: Glycogen debranching enzyme     Goto   Sponge   NotDistinct   Permalink

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A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen, which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. Together with phosphorylases, debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result.

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  • Glykogen-Debranching-Enzym (de)
  • Enzima desramificadora del glucógeno (es)
  • Glycogen debranching enzyme (en)
  • Enzyme de débranchement du glycogène (fr)
  • Аміло-1,6-глюкозидаза (uk)
rdfs:comment
  • Glykogen-debranching-Enzym (Gen: AGL) heißt das Enzym, das Verzweigungen im Glykogen abbaut. Der Abbau dieser Verzweigungen ist ein Teilschritt bei der Verwertung des Reservestoffs Glykogen im Rahmen der Glykogenolyse. Das Enzym kommt in Säugetieren und Pilzen vor. (de)
  • A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen, which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. Together with phosphorylases, debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. (en)
  • La enzima desramificadora del glucógeno (AGL) participa en la glucogenólisis desramificando las cadenas de glucógeno mediante las reacciones:​​ * Transferencia de un segmento de (1→4)-alfa-D-glucano a una nueva posición en un aceptor, que puede ser glucosa o un (1→4)-alfa-D-glucano. * Hidrólisis de un enlace ramificado (1→6)-alfa-D-glucosídico del glucógeno. La enzima incluye dos dominios, cada uno de ellos para las reacciones antes citadas. Es decir, tiene un dominio 4-alfa-glucanotransferasa (EC 2.4.1.25) y un dominio amilo-alfa-1,6-glucosidasa (EC 3.2.1.33). (es)
  • L'enzyme de débranchement du glycogène (GDE) est un système enzymatique regroupant une activité glycosyltransférase et une activité glycoside hydrolase qui facilite la dégradation du glycogène stocké dans le foie et les muscles en libérant du glucose avec l'aide de phosphorylases. Il s'agit d'une source d'énergie essentielle chez de nombreux êtres vivants. Cette voie métabolique est régulée dans l'organisme, particulièrement dans le foie, sous l'action de diverses hormones telles que l'insuline et le glucagon. Cette régulation très étroite assure l'homéostasie de la glycémie. Les désordres métaboliques résultant de mutations affectant l'enzyme de débranchement du glycogène peuvent conduire par exemple à une glycogénose type 3. (fr)
  • AGL (англ. Amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 1-ї хромосоми. Довжина поліпептидного ланцюга білка становить 1 532 амінокислот, а молекулярна маса — 174 764. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин (uk)
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  • http://commons.wikimedia.org/wiki/Special:FilePath/Function_and_Structure_of_Eukaryotic_Glycogen_Debranching_Enzyme.jpg
  • http://commons.wikimedia.org/wiki/Special:FilePath/Glycosidase_mechanism.png
  • http://commons.wikimedia.org/wiki/Special:FilePath/Hypothesized_substraight_binding_location.png
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