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1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene. Glycogen branching enzyme is an enzyme that adds branches to the growing glycogen molecule during the synthesis of glycogen, a storage form of glucose. More specifically, during glycogen synthesis, a glucose 1-phosphate molecule reacts with uridine triphosphate (UTP) to become UDP-glucose, an activated form of glucose. The activated glucosyl unit of UDP-glucose is then transferred to the hydroxyl group at the C-4 of a terminal residue of glycogen to form an α-1,4-glycosidic linkage, a reaction catalyzed by glycogen synthase. Importantly, glycogen synthase can only catalyze the synthesis of α-1,4-glycosidic linkages. Since glycogen is a readi

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  • La enzima ramificadora del glucógeno (GBE1) (EC 2.4.1.18) cataliza la reacción de adición de una rama de glucosas al polímero glucógeno en formación mediante un enlace α-1,6-glucosídico.​ (es)
  • 1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene. Glycogen branching enzyme is an enzyme that adds branches to the growing glycogen molecule during the synthesis of glycogen, a storage form of glucose. More specifically, during glycogen synthesis, a glucose 1-phosphate molecule reacts with uridine triphosphate (UTP) to become UDP-glucose, an activated form of glucose. The activated glucosyl unit of UDP-glucose is then transferred to the hydroxyl group at the C-4 of a terminal residue of glycogen to form an α-1,4-glycosidic linkage, a reaction catalyzed by glycogen synthase. Importantly, glycogen synthase can only catalyze the synthesis of α-1,4-glycosidic linkages. Since glycogen is a readily mobilized storage form of glucose, the extended glycogen polymer is branched by glycogen branching enzyme to provide glycogen breakdown enzymes, such as glycogen phosphorylase, with many terminal residues for rapid degradation. Branching also importantly increases the solubility and decreases the osmotic strength of glycogen. The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. The highest levels of this enzyme are found in liver and muscle cells. Mutations in this gene are associated with glycogen storage disease type IV (also known as Andersen's disease). (en)
  • L'enzima ramificante dell'1,4-alfa-glucano è un enzima appartenente alla classe delle transferasi, che catalizza la seguente reazione: Trasferimento di un segmento di un 1,4-α glucano ad un gruppo idrossi di un in una catena simile Converte l'amilosio in amilopectina. Il nome accettato richiede una qualifica a seconda dei prodotti, il glicogeno e amilopectina, ad esempio enzima ramificante del glicogeno, enzima ramificante dell'amilopectina. Quest'ultimo è stato spesso chiamato Q-enzima. (it)
  • 1,4-alfaglukandelningsenzym, även känt som branching enzyme är ett enzym involverat i syntesen av glykogen i kroppens celler. Det överför en kedja med 1-4-alfaglykanbindningar till en annan kedjas primära hydroxigrupp, vilket skapar en 1-6-binding. Enzymet sätter en förgrening vid var 10:e glukosrest på glykogenmolekylen. (sv)
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  • Glycogen branching enzyme (en)
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  • La enzima ramificadora del glucógeno (GBE1) (EC 2.4.1.18) cataliza la reacción de adición de una rama de glucosas al polímero glucógeno en formación mediante un enlace α-1,6-glucosídico.​ (es)
  • L'enzima ramificante dell'1,4-alfa-glucano è un enzima appartenente alla classe delle transferasi, che catalizza la seguente reazione: Trasferimento di un segmento di un 1,4-α glucano ad un gruppo idrossi di un in una catena simile Converte l'amilosio in amilopectina. Il nome accettato richiede una qualifica a seconda dei prodotti, il glicogeno e amilopectina, ad esempio enzima ramificante del glicogeno, enzima ramificante dell'amilopectina. Quest'ultimo è stato spesso chiamato Q-enzima. (it)
  • 1,4-alfaglukandelningsenzym, även känt som branching enzyme är ett enzym involverat i syntesen av glykogen i kroppens celler. Det överför en kedja med 1-4-alfaglykanbindningar till en annan kedjas primära hydroxigrupp, vilket skapar en 1-6-binding. Enzymet sätter en förgrening vid var 10:e glukosrest på glykogenmolekylen. (sv)
  • 1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene. Glycogen branching enzyme is an enzyme that adds branches to the growing glycogen molecule during the synthesis of glycogen, a storage form of glucose. More specifically, during glycogen synthesis, a glucose 1-phosphate molecule reacts with uridine triphosphate (UTP) to become UDP-glucose, an activated form of glucose. The activated glucosyl unit of UDP-glucose is then transferred to the hydroxyl group at the C-4 of a terminal residue of glycogen to form an α-1,4-glycosidic linkage, a reaction catalyzed by glycogen synthase. Importantly, glycogen synthase can only catalyze the synthesis of α-1,4-glycosidic linkages. Since glycogen is a readi (en)
rdfs:label
  • 1,4-α-Glucan-verzweigendes Enzym (de)
  • Glycogen branching enzyme (en)
  • Enzima ramificadora del glucógeno (es)
  • 1,4-alfa-glucano enzima ramificante (it)
  • 1,4-alfaglukandelningsenzym (sv)
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