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Western Blot (WB) (Western Blot (WB) analysis of GBE1 polyclonal antibody)

Rabbit GBE1 Polyclonal Antibody | anti-GBE1 antibody

GBE1 Polyclonal Antibody

Gene Names
GBE1; GBE; APBD; GSD4
Reactivity
Human, Mouse, Rat
Applications
Western Blot
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
Synonyms
GBE1; Polyclonal Antibody; GBE1 Polyclonal Antibody; 1; 4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme; anti-GBE1 antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human, Mouse, Rat
Clonality
Polyclonal
Isotype
IgG
Specificity
GBE1 polyclonal antibody detects endogenous levels of GBE1 protein.
Purity/Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
Form/Format
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Sequence Length
702
Applicable Applications for anti-GBE1 antibody
Western Blot (WB)
Application Notes
WB: 1:500-1:2000
Immunogen
Recombinant full length Human GBE1.
Preparation and Storage
Store at 4 degree C short term.
Aliquot and store at -20 degree C long term.
Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of GBE1 polyclonal antibody)

Western Blot (WB) (Western Blot (WB) analysis of GBE1 polyclonal antibody)
Related Product Information for anti-GBE1 antibody
GBE1 (glucan (1, 4-alpha-), branching enzyme 1) is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 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 a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
UniProt Accession #
Molecular Weight
~ 80kDa
NCBI Official Full Name
1,4-alpha-glucan-branching enzyme
NCBI Official Synonym Full Names
1,4-alpha-glucan branching enzyme 1
NCBI Official Symbol
GBE1
NCBI Official Synonym Symbols
GBE; APBD; GSD4
NCBI Protein Information
1,4-alpha-glucan-branching enzyme
UniProt Protein Name
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

Research Articles on GBE1

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Product Notes

The GBE1 gbe1 (Catalog #AAA3011618) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GBE1 Polyclonal Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GBE1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500-1:2000. Researchers should empirically determine the suitability of the GBE1 gbe1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GBE1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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