AGL Recombinant Monoclonal Antibody

Datasheet

Code	CSB-RA116104A0HU
Size	US$210
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Image	Western Blot Positive WB detected in: LO2 whole cell lysate(30µg), Rat skeletal muscle tissue lysate(30µg), Mouse tongue tissue lysate(30µg) All lanes: AGL antibody at 1:1000 Secondary Goat polyclonal to rabbit IgG at 1/40000 dilution Predicted band size: 175 kDa Observed band size: 175 kDa Exposure time：2min IHC image of CSB-RA116104A0HU diluted at 1:100 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Overlay Peak curve showing Hela cells stained with CSB-RA116104A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/110⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/110⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
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Target Background

Product Details

Uniprot No.

P35573

Target Names

AGL

Alternative Names

Glycogen debranching enzyme (Glycogen debrancher) [Includes: 4-alpha-glucanotransferase (EC 2.4.1.25) (Oligo-1,4-1,4-glucantransferase), Amylo-alpha-1,6-glucosidase (Amylo-1,6-glucosidase) (EC 3.2.1.33) (Dextrin 6-alpha-D-glucosidase)], AGL, GDE

Species Reactivity

Human, Mouse, Rat

Immunogen

Recombinant Antibody

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

8D4

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid

Tested Applications

ELISA, WB, IHC, FC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:50-1:200
FC	1:50-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

AGL, also known as glycogen debranching enzyme, plays an essential role in glycogen metabolism by catalyzing two distinct enzymatic activities: 4-alpha-glucanotransferase and amylo-alpha-1,6-glucosidase. This bifunctional enzyme is critical for the complete breakdown of glycogen, and mutations in AGL are associated with glycogen storage disease type III, making it a significant target for metabolic disease research, liver pathology studies, and investigations into muscle disorders.

This recombinant monoclonal antibody, clone 8D4, offers the reproducibility and consistency that demanding research workflows require. Produced using recombinant technology with a defined sequence, this antibody eliminates the lot-to-lot variability that can complicate long-term studies or multi-site collaborations. The rabbit IgG format and affinity-chromatography purification ensure high specificity for your target.

Validation studies demonstrate robust performance across multiple applications and sample types. Western blot analysis confirms detection of AGL at the expected 175 kDa molecular weight in human LO2 whole cell lysate, rat skeletal muscle tissue, and mouse tongue tissue, providing researchers with confidence when working across these species. Immunohistochemistry validation in paraffin-embedded human liver tissue shows clear staining using standard antigen retrieval protocols, while flow cytometry analysis in HeLa cells demonstrates effective intracellular detection with appropriate signal separation from isotype controls.

The versatility across ELISA, immunohistochemistry, and flow cytometry applications gives researchers flexibility to investigate AGL expression and localization using complementary approaches. Whether you are studying glycogen metabolism, characterizing metabolic disorders, or examining tissue-specific expression patterns in liver and muscle, this antibody provides a reliable tool for advancing your research.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function

Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4-alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6-glucosidase in glycogen degradation.

Gene References into Functions

This report of patients with GSD-III in Iran with 2 uncommon clinical presentations and 5 novel mutations in the AGL gene. PMID: 29794575
The study identified 31 novel mutations and extended the mutation spectrum of AGL in Chinese patients with glycogen storage disease type III. PMID: 26984562
Our study establishes HAS2-mediated HA synthesis as a driver of growth of bladder cancer with low AGL and provides preclinical rationale for personalized targeting of HAS2/HA signaling in patients with low amylo-alpha-1-6-glucosidase-4-alpha-glucanotransferase -expressing tumors. PMID: 26490312
AGL loss causes high SHMT2 expression and consequently increases glycine-dependent nucleotide synthesis leading to bladder cancer growth. PMID: 26975021
Point mutations in AGL gene are associated with glycogen storage disease type IIIa in a Chinese family. PMID: 26252094
Haplotype analysis revealed that the mutation arises as a result of founder effect, not an independent event. PMID: 25827695
AGL haplotype analyses suggested that c.1019delA and c.958+1G>A are founder mutations in Turkish patients, while p.R864X is a recurrent mutation. PMID: 25451950
A homozygous frameshift deletion, c.4456delT, in exon 33 of the AGL gene in Inuit children determines the cause of glycogen storage disease type IIIa and confirms a founder effect. PMID: 25602008
study identified 10 different mutations in 8 Korean Glycogen storage disease type III patients; 5 mutations are novel and include 1 nonsense (c.1461G>A, p.W487X), 3 splicing (c.293+4_293+6delAGT in IVS4, c.460+1G>T in IVS5, c.2682-8A>G in IVS21) and 1 missense mutation (c.2591G>C, p.R864P) PMID: 24257475
Characterization of a novel homozygous single point mutation at the polypyrimidine tract of intron 21 of the AGL gene in two consanguineous siblings with glycogen storage disease type III. PMID: 23649758
We found that most patients with macular telangiectasia-2 possess retinal autoantibodies, the most prevalent of which were directed against AGL, RBP3, and CK-B. PMID: 23882694
A founder effect discovered amongst Tunisian patients with glycogen storage disease type III and a c.3216_3217delGA mutation in the AGL gene. PMID: 22035446
Mutations in amylo-1,6-glucosidase is associated with Glycogen Storage Disease Type III. PMID: 20648714
The present patient was found to be deficient in GDE activity and homozygous for a novel 1 bp deletion in AGL. This mutation is predicted to cause premature termination at codon 834 due to frame shift. PMID: 20158661
GSD-III patients have variable phenotypic characteristics. Administration of raw-corn-starch can effectively improve the disease outcome. We identified 8 new mutations on AGL gene through nucleotide sequence analysis. PMID: 15833157
Nine AGL mutations: six nonsense mutations , one deletion and two splicing mutation were identified in Turkish GSD III patients. PMID: 19834502
Mutations associated with GSD III include R34X and Y1148X. PMID: 11924557
it is likely that the AMPK-GDE association is a novel mechanism regulating AMPK activity and the resultant fatty acid oxidation and glucose uptake PMID: 15886229
AGL gene mutations may have roles in glycogen storage disease type III PMID: 17047887
These results indicate that binding to glycogen crucially regulates the stability of AGL and, further, that its ubiquitination may play an important role in the pathophysiology of both Lafora and Cori's disease. PMID: 17908927
Current clinical and molecular knowledge about glycogenosis 3 and phenotype and genotype levels of this enzyme. [REVIEW] PMID: 17915576
a homozygous p.W1327X mutation leads to severe generalized glycogenosis types 3a & 3b within the same family; heterozygous p.W1327X mutation carriers may present with mild non-progressive neuromuscular symptoms, such as exercise-induced myalgia & fatigue PMID: 18924225
Mutations in the carbohydrate-binding domain of AGL lead to loss of all enzymatic activities and enhancing targeting for proteasomal degradation. PMID: 19299494
Six novel AGL mutations were identified. PMID: 19754354

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Involvement in disease

Glycogen storage disease 3 (GSD3)

Subcellular Location

Cytoplasm. Note=Under glycogenolytic conditions localizes to the nucleus.

Protein Families

Glycogen debranching enzyme family

Tissue Specificity

Liver, kidney and lymphoblastoid cells express predominantly isoform 1; whereas muscle and heart express not only isoform 1, but also muscle-specific isoform mRNAs (isoforms 2, 3 and 4). Isoforms 5 and 6 are present in both liver and muscle.

Database Links

HGNC: 321

OMIM: 232400

KEGG: hsa:178

STRING: 9606.ENSP00000294724

UniGene: Hs.904

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

No. 269, Shendun 5th Road, Donghu Hi-Tech Development Area, Hubei Province, 430206, P.R.China

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