Rabbit anti-Human, Mouse GBE1 Polyclonal Antibody | anti-GBE1 antibody

GBE1, ID (GBE1, 1,4-alpha-glucan-branching enzyme, Brancher enzyme, Glycogen-branching enzyme) (Azide free) (HRP)

Cat. Num. AAA6301438

• Gene Names: GBE1; GBE; APBD; GSD4
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: GBE1; Polyclonal Antibody; ID (GBE1; 1; 4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme) (Azide free) (HRP); Glycogen-branching enzyme; anti-GBE1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).

• Applicable Applications for anti-GBE1 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: GBE1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 534~564 amino acids from the Central region of human GBE1.
• Conjugate: HRP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of GBE1 Antibody (Center) in HL-60 cell line lysates (35ug/lane). GBE1 (arrow) was detected using the purified Pab;)

Western Blot (WB)

(Western blot analysis of GBE1 Antibody (Center) in mouse liver tissue lysates (35ug/lane).GBE1 (arrow) was detected using the purified Pab.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of GBE1 Antibody (Center) with HepG2 cell followed by Alexa Fluor? 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red). DAPI was used to stain the cell nuclear (blue).)

Related Product Information for anti-GBE1 antibody

GBE1 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).

Product Categories/Family for anti-GBE1 antibody

Antibodies; Proteins

References

Konstantinidou, A.E., et al. Placenta 29(4):378-381(2008). Massa, R., et al. Muscle Nerve 37(4):530-536(2008). Bruno, C., et al. Acta Myol 26(1):75-78(2007).

NCBI and Uniprot Product Information

• NCBI GI #: 357529509
• NCBI GeneID: 2632
• UniProt Accession #: Q04446; Q96EN0; B3KWV3
• Molecular Weight: 702
• NCBI Official Full Name: 1,4-alpha-glucan-branching enzyme
• NCBI Official Synonym Full Names: glucan (1,4-alpha-), branching enzyme 1
• NCBI Official Symbol: GBE1
• NCBI Official Synonym Symbols: GBE; APBD; GSD4
• NCBI Protein Information: 1,4-alpha-glucan-branching enzyme; brancher enzyme; glycogen branching enzyme; glycogen-branching enzyme; amylo-(1,4 to 1,6) transglucosidase; amylo-(1,4 to 1,6) transglycosylase
• UniProt Protein Name: 1,4-alpha-glucan-branching enzyme
• Protein Family: 1,4-alpha-glucan branching enzyme
• UniProt Gene Name: GBE1
• UniProt Entry Name: GLGB_HUMAN

NCBI Description

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]

Uniprot Description

GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen's disease).

Protein type: EC 2.4.1.18; Carbohydrate Metabolism - starch and sucrose; Transferase

Chromosomal Location of Human Ortholog: 3p12.3

Cellular Component: cytosol

Molecular Function: cation binding; 1,4-alpha-glucan branching enzyme activity; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: glycogen metabolic process; generation of precursor metabolites and energy; glycogen biosynthetic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis

Disease: Polyglucosan Body Disease, Adult Form; Glycogen Storage Disease Iv