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GBE1 Produits

(Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Catégories

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. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008].

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Catégories GBE1 en vedette

GBE1 Anticorps

High quality antibodies with extensive validation data.

GBE1 Kits ELISA

Reliable ELISA kits for a wide range of species.

GBE1 Protéines

Proteins for various applications incl. WB, ELISA, IF etc.

GBE1 recommandé Anticorps

Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Application WB, IHC, IP, FACS, IHC (p)
Validations
  • (9)
N° du produit ABIN1498396
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Human
Application WB, IF, IP, FACS
Validations
  • (4)
N° du produit ABIN1498395
Quantité 100 μL
Fiche technique Fiche technique
Reactivity Human, Mouse
Application WB, IF
Validations
  • (3)
N° du produit ABIN653459
Quantité 400 μL
Fiche technique Fiche technique

GBE1 recommandé Kits ELISA

Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Analytical Method Quantitative Competition ELISA
Validations
N° du produit ABIN1742631
Quantité 96 tests
Fiche technique Fiche technique
Reactivity Sheep
Analytical Method
Validations
N° du produit ABIN1773535
Quantité 96 tests
Fiche technique Fiche technique
Reactivity Pig
Analytical Method
Validations
N° du produit ABIN1749960
Quantité 96 tests
Fiche technique Fiche technique

GBE1 recommandé Protéines

Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1354871
Quantité 10 μg
Fiche technique Fiche technique
Reactivity Human
Source Wheat germ
Validations
  • (1)
N° du produit ABIN1354873
Quantité 10 μg
Fiche technique Fiche technique
Reactivity Human
Source Insect Cells
Validations
  • (1)
N° du produit ABIN3080739
Quantité 1 mg
Fiche technique Fiche technique

Dernières publications sur nos produits GBE1

Colombo, Pagliarani, Testolin, Salsano, Napoli, Bordoni, Salani, DAdda, Morandi, Farina, Magri, Riva, Prelle, Sciacco, Comi, Moggio: "Adult polyglucosan body disease: clinical and histological heterogeneity of a large Italian family." dans: Neuromuscular disorders : NMD, Vol. 25, Issue 5, pp. 423-8, (2015) (PubMed).

Irimia, Tagliabracci, Meyer, Segvich, DePaoli-Roach, Roach: "Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice." dans: The Journal of biological chemistry, (2015) (PubMed).

Orhan Akman, Emmanuele, Kurt, Kurt, Sheiko, DiMauro, Craigen: "A novel mouse model that recapitulates adult-onset glycogenosis type 4." dans: Human molecular genetics, Vol. 24, Issue 23, pp. 6801-10, (2015) (PubMed).

Kakhlon, Glickstein, Feinstein, Liu, Baba, Terashima, Akman, Dimauro, Lossos: "Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase." dans: Journal of neurochemistry, Vol. 127, Issue 1, pp. 101-13, (2013) (PubMed).

Akman, Sheiko, Tay, Finegold, Dimauro, Craigen: "Generation of a novel mouse model that recapitulates early and adult onset glycogenosis type IV." dans: Human molecular genetics, Vol. 20, Issue 22, pp. 4430-9, (2011) (PubMed).

Synonymes et noms alternatifs relatifs à GBE1

1,4-alpha-glucan branching protein GlgB (glgB), 1,4-alpha-glucan branching protein (glgB), glycogen branching protein (ECs4277), glycogen branching protein (glgB), glycogen branching enzyme (glgB), 1,4-alpha-glucan-branching protein (SCO7332), glycogen branching enzyme; BE; 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-glucosyl-transferase (glgB), glycogen branching enzyme (Synpcc7942_1085), glycogen branching enzyme (AZC_4116), glycogen branching enzyme (Sros_8169), glycogen branching enzyme (Despr_0975), 1,4-alpha-glucan branching enzyme 1 (GBE1), glucan (1,4-alpha-), branching enzyme 1 (Gbe1), 1,4-alpha-glucan branching enzyme 1 (Gbe1), 2310045H19Rik, 2810426P10Rik, APBD, D16Ertd536e, GBE, glgBII, GSD4

Protein level used designations for GBE1

  • glycogen branching enzyme
  • 1,4-alpha-glucan-branching enzyme
  • amylo-(1,4 to 1,6) transglucosidase
  • amylo-(1,4 to 1,6) transglycosylase
  • brancher enzyme
  • glycogen-branching enzyme
  • glucan (1,4-alpha-), branching enzyme 1 (glycogen branching enzyme, Andersen disease, glycogen storage disease type IV)
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