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GBE1 Products

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

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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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Featured GBE1 Categories

GBE1 Antibodies

High quality antibodies with extensive validation data.

GBE1 ELISA Kits

Reliable ELISA kits for a wide range of species.

GBE1 Proteins

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

Recommended GBE1 Antibodies

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, IHC, IP, FACS, IHC (p)
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  • (9)
Cat. No. ABIN1498396
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application WB, IF, IP, FACS
Validations
  • (4)
Cat. No. ABIN1498395
Quantity 100 μL
Datasheet Datasheet
Reactivity Human, Mouse
Application WB, IF
Validations
  • (3)
Cat. No. ABIN653459
Quantity 400 μL
Datasheet Datasheet

Recommended GBE1 ELISA Kits

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Mouse
Analytical Method
Validations
Cat. No. ABIN1745072
Quantity 96 tests
Datasheet Datasheet
Reactivity Cow
Analytical Method
Validations
Cat. No. ABIN1771087
Quantity 96 tests
Datasheet Datasheet
Reactivity Rat
Analytical Method Quantitative Competition ELISA
Validations
Cat. No. ABIN1743854
Quantity 96 tests
Datasheet Datasheet

Recommended GBE1 Proteins

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
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Cat. No. ABIN1354871
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
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  • (1)
Cat. No. ABIN1354873
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Insect Cells
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Cat. No. ABIN3080739
Quantity 1 mg
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Latest Publications for our GBE1 products

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." in: 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." in: 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." in: 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." in: 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." in: Human molecular genetics, Vol. 20, Issue 22, pp. 4430-9, (2011) (PubMed).

Synonyms and alternative names related to 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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