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
Overview
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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.
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Positive control
- Human fetal liver, PC-3 or Human skeletal muscle lysate, HeLa cells.
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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
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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...
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Purity
Tissue culture supernatant -
Clonality
Monoclonal -
Clone number
EP11113 -
Isotype
IgG -
Research areas
Associated products
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Isotype control
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 |
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WB |
1/1000 - 1/10000. Detects a band of approximately 70 kDa.
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ICC/IF |
1/100.
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Flow Cyt |
1/140.
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
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Notes |
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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.
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Target
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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. - Information by UniProt
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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
see all -
Alternative names
- 1 antibody
- 1,4 alpha glucan branching enzyme antibody
- 1,4-alpha-glucan branching enzyme 1 antibody
see all
Images
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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 -
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 -
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.
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Flow cytometry analysis of HeLa cells using ab180596 at a 1/40 dilution (red) and a rabbit IgG as negative control (green).
Protocols
Datasheets and documents
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SDS download
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Datasheet download
Certificate of Compliance
References (4)
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