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Signal Transduction Metabolism Energy Metabolism
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RabMAb

Anti-GBE1 antibody [EP11113] (ab180596)

  • Datasheet
  • SDS
  • Certificate of Compliance
Submit a review Submit a question References (4)

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Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
  • Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
  • Immunocytochemistry/ Immunofluorescence - Anti-GBE1 antibody [EP11113] (ab180596)
  • Flow Cytometry - Anti-GBE1 antibody [EP11113] (ab180596)

Key features and details

  • Rabbit monoclonal [EP11113] to GBE1
  • Suitable for: WB, ICC/IF, Flow Cyt
  • Reacts with: Human
  • Isotype: IgG

You may also be interested in

Secondary
Product image
Goat Anti-Rabbit IgG H&L (HRP) (ab205718)

View more associated products

Overview

  • Product name

    Anti-GBE1 antibody [EP11113]
    See all GBE1 primary antibodies
  • Description

    Rabbit monoclonal [EP11113] to GBE1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IF, Flow Cytmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Horse, Cat
  • Immunogen

    Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.

  • Positive control

    • Human fetal liver, PC-3 or Human skeletal muscle lysate, HeLa cells.
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

    The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

    If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.20
    Preservative: 0.01% Sodium azide
    Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant
  • Concentration information loading...
  • Purity

    Tissue culture supernatant
  • Clonality

    Monoclonal
  • Clone number

    EP11113
  • Isotype

    IgG
  • Research areas

    • Signal Transduction
    • Metabolism
    • Energy Metabolism
    • Cancer
    • Cancer Metabolism
    • Metabolic signaling pathway
    • Metabolism of carbohydrates
    • Metabolism
    • Pathways and Processes
    • Metabolic signaling pathways
    • Carbohydrate metabolism
    • Metabolism
    • Pathways and Processes
    • Metabolic signaling pathways
    • Energy transfer pathways
    • Energy Metabolism

Associated products

  • Isotype control

    • Rabbit IgG, monoclonal [EPR25A] - Isotype Control (ab172730)

Applications

The Abpromise guarantee

Our Abpromise guarantee covers the use of ab180596 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB
1/1000 - 1/10000. Detects a band of approximately 70 kDa.
ICC/IF
1/100.
Flow Cyt
1/140.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

 

Notes
WB
1/1000 - 1/10000. Detects a band of approximately 70 kDa.
ICC/IF
1/100.
Flow Cyt
1/140.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

 

Target

  • Function

    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.
  • Tissue specificity

    Highest levels found in liver and muscle.
  • Pathway

    Glycan biosynthesis; glycogen biosynthesis.
  • Involvement in disease

    Defects in GBE1 are the cause of glycogen storage disease type 4 (GSD4) [MIM:232500]; also known as Andersen disease. GSD4 is a metabolic disorder characterized by the accumulation of an amylopectin-like polysaccharide. The typical clinical manifestation is liver disease of childhood, progressing to lethal hepatic cirrhosis. Most children with this condition die before two years of age. However, the liver disease is not always progressive. No treatment apart from liver transplantation has been found to prevent progression of the disease. There is also a neuromuscular form of GSD4 that varies in onset (perinatal, congenital, juvenile, or adult) and severity.
    Note=Neuromuscular perinatal glycogen storage disease type 4 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
    Defects in GBE1 are the cause of adult polyglucosan body disease (APBD) [MIM:263570]. APBD is a late-onset, slowly progressive disorder affecting the central and peripheral nervous systems. Patients typically present after age 40 years with a variable combination of cognitive impairment, pyramidal tetraparesis, peripheral neuropathy, and neurogenic bladder. Other manifestations include cerebellar dysfunction and extrapyramidal signs. The pathologic hallmark of APBD is the widespread accumulation of round, intracellular polyglucosan bodies throughout the nervous system, which are confined to neuronal and astrocytic processes.
  • Sequence similarities

    Belongs to the glycosyl hydrolase 13 family.
  • Target information above from: UniProt accession Q04446 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links

    • Entrez Gene: 493962 Cat
    • Entrez Gene: 100034152 Horse
    • Entrez Gene: 2632 Human
    • Entrez Gene: 74185 Mouse
    • Entrez Gene: 288333 Rat
    • Omim: 607839 Human
    • SwissProt: Q6T308 Cat
    • SwissProt: Q6EAS5 Horse
    • SwissProt: Q04446 Human
    • SwissProt: Q9D6Y9 Mouse
    • Unigene: 436062 Human
    • Unigene: 396102 Mouse
    • Unigene: 29938 Rat
    see all
  • Alternative names

    • 1 antibody
    • 1,4 alpha glucan branching enzyme antibody
    • 1,4-alpha-glucan branching enzyme 1 antibody
    • 4-alpha-glucan-branching enzyme antibody
    • amylo (1,4 to 1,6) transglucosidase antibody
    • amylo (1,4 to 1,6) transglycosylase antibody
    • Andersen disease antibody
    • APBD antibody
    • Brancher enzyme antibody
    • GBE 1 antibody
    • GBE antibody
    • GBE1 antibody
    • gGlucan (1,4 alpha ), branching enzyme 1 antibody
    • GLGB_HUMAN antibody
    • Glucan (1,4 alpha) branching enzyme antibody
    • Glycogen branching enzyme antibody
    • Glycogen storage disease type IV antibody
    • Glycogen-branching enzyme antibody
    • GSD4 antibody
    • OTTHUMP00000213788 antibody
    • OTTHUMP00000213833 antibody
    see all

Images

  • Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
    Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
    All lanes : Anti-GBE1 antibody [EP11113] (ab180596) at 1/10000 dilution

    Lane 1 : Human fetal liver lysate
    Lane 2 : PC-3 lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG (H+L) Peroxidase conjugated at 1/1000 dilution
  • Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
    Western blot - Anti-GBE1 antibody [EP11113] (ab180596)
    Anti-GBE1 antibody [EP11113] (ab180596) at 1/2000 dilution + Human skeletal muscle lysate at 10 µg

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) (ab136636) at 1/500 dilution
  • Immunocytochemistry/ Immunofluorescence - Anti-GBE1 antibody [EP11113] (ab180596)
    Immunocytochemistry/ Immunofluorescence - Anti-GBE1 antibody [EP11113] (ab180596)

    Immunofluorescence analysis of HeLa cells (fixative 4% paraformaldehyde) labeling GBE1 with ab180596 at a 1/100 dilution (left image), and counterstained with Dapi (right image). Goat anti rabbit IgG (Dylight 555) secondary used at a 1/200 diution.

  • Flow Cytometry - Anti-GBE1 antibody [EP11113] (ab180596)
    Flow Cytometry - Anti-GBE1 antibody [EP11113] (ab180596)

    Flow cytometry analysis of HeLa cells using ab180596 at a 1/40 dilution (red) and a rabbit IgG as negative control (green).

Protocols

  • Flow cytometry protocols
  • Immunocytochemistry & immunofluorescence protocols
  • Western blot protocols

Click here to view the general protocols

Datasheets and documents

  • SDS download

  • Datasheet download

    Download

Certificate of Compliance

To download a Certificate of Compliance, please enter your Lot number below:

References (4)

Publishing research using ab180596? Please let us know so that we can cite the reference in this datasheet.

ab180596 has been referenced in 4 publications.

  • Li L  et al. Hypoxia-induced GBE1 expression promotes tumor progression through metabolic reprogramming in lung adenocarcinoma. Signal Transduct Target Ther 5:54 (2020). PubMed: 32439898
  • Sullivan MA  et al. Skeletal Muscle Glycogen Chain Length Correlates with Insolubility in Mouse Models of Polyglucosan-Associated Neurodegenerative Diseases. Cell Rep 27:1334-1344.e6 (2019). PubMed: 31042462
  • Uruno A  et al. Nrf2-Mediated Regulation of Skeletal Muscle Glycogen Metabolism. Mol Cell Biol 36:1655-72 (2016). PubMed: 27044864
  • Krag TO  et al. Differential glucose metabolism in mice and humans affected by McArdle disease. Am J Physiol Regul Integr Comp Physiol 311:R307-14 (2016). PubMed: 27280431

Customer reviews and Q&As

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