AGL Antibody (B-11): sc-518176
- AGL Antibody (B-11) is a mouse monoclonal IgG1 κ provided at 200 µg/ml
- raised against amino acids 1233-1532 mapping at the C-terminus of AGL of human origin
- recommended for detection of AGL of mouse, rat and human origin by WB, IP, IF and ELISA
- Anti-AGL Antibody (B-11) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP, m-IgG1 BP-HRP and m-IgGκ BP-HRP are the preferred secondary detection reagents for AGL Antibody (B-11) for WB applications. These reagents are now offered in bundles with AGL Antibody (B-11) (see ordering information below).
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SEE ALSO...
AGL Antibody (B-11) is a mouse monoclonal IgG1 kappa light chain antibody that detects AGL protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-AGL antibody (B-11) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. AGL, or amylo-1,6-glucosidase, 4-alpha-glucanotransferase, is a crucial 1,532 amino acid protein that plays a vital role in glycogen metabolism, existing as three alternatively spliced isoforms expressed in kidney, liver, heart, and muscle tissues in an isoform-specific manner. AGL′s multifunctional enzyme capabilities are significant, as AGL contains two catalytic active sites; one functions as a 4-alpha-glucotransferase, while the other acts as an amylo-1,6-glucosidase, facilitating the breakdown of glycogen into glucose. This enzymatic activity is essential for maintaining normal blood sugar levels and energy homeostasis. Defects in the gene encoding AGL lead to glycogen storage disease type 3 (GSD3), also known as Forbes disease, a metabolic disorder characterized by abnormal glycogen accumulation due to insufficient AGL activity. Symptoms of GSD3 typically include hypoglycemia, variable myopathy, hepatomegaly, and short stature, underscoring the importance of AGL in metabolic health.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
AGL Antibody (B-11) References:
- Mutational and haplotype analysis of AGL in patients with glycogen storage disease type III. | Horinishi, A., et al. 2002. J Hum Genet. 47: 55-9. PMID: 11924557
- Molecular cloning and nucleotide sequence of cDNA encoding human muscle glycogen debranching enzyme. | Yang, BZ., et al. 1992. J Biol Chem. 267: 9294-9. PMID: 1374391
- Glycogen debranching enzyme association with beta-subunit regulates AMP-activated protein kinase activity. | Sakoda, H., et al. 2005. Am J Physiol Endocrinol Metab. 289: E474-81. PMID: 15886229
- Molecular analysis of the AGL gene: heterogeneity of mutations in patients with glycogen storage disease type III from Germany, Canada, Afghanistan, Iran, and Turkey. | Endo, Y., et al. 2006. J Hum Genet. 51: 958-963. PMID: 17047887
- A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease. | Cheng, A., et al. 2007. Genes Dev. 21: 2399-409. PMID: 17908927
- Clinical, biochemical and genetic features of glycogen debranching enzyme deficiency. | Lucchiari, S., et al. 2007. Acta Myol. 26: 72-4. PMID: 17915576
- Immunoblot analyses of glycogen debranching enzyme in different subtypes of glycogen storage disease type III. | Ding, JH., et al. 1990. J Pediatr. 116: 95-100. PMID: 2295969
- Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle. | Shen, J., et al. 1996. J Clin Invest. 98: 352-7. PMID: 8755644
- Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0. | Orho, M., et al. 1998. J Clin Invest. 102: 507-15. PMID: 9691087
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
AGL Antibody (B-11) | sc-518176 | 200 µg/ml | $322.00 | |||
AGL Antibody (B-11): m-IgG Fc BP-HRP Bundle | sc-550701 | 200 µg Ab; 10 µg BP | $361.00 | |||
AGL Antibody (B-11): m-IgGκ BP-HRP Bundle | sc-550700 | 200 µg Ab; 40 µg BP | $361.00 | |||
AGL Antibody (B-11): m-IgG1 BP-HRP Bundle | sc-550702 | 200 µg Ab; 20 µg BP | $361.00 | |||
AGL Antibody (B-11) AC | sc-518176 AC | 500 µg/ml, 25% agarose | $424.00 | |||
AGL Antibody (B-11) HRP | sc-518176 HRP | 200 µg/ml | $322.00 | |||
AGL Antibody (B-11) FITC | sc-518176 FITC | 200 µg/ml | $336.00 | |||
AGL Antibody (B-11) PE | sc-518176 PE | 200 µg/ml | $349.00 | |||
AGL Antibody (B-11) Alexa Fluor® 488 | sc-518176 AF488 | 200 µg/ml | $364.00 | |||
AGL Antibody (B-11) Alexa Fluor® 546 | sc-518176 AF546 | 200 µg/ml | $364.00 | |||
AGL Antibody (B-11) Alexa Fluor® 594 | sc-518176 AF594 | 200 µg/ml | $364.00 | |||
AGL Antibody (B-11) Alexa Fluor® 647 | sc-518176 AF647 | 200 µg/ml | $364.00 | |||
AGL Antibody (B-11) Alexa Fluor® 680 | sc-518176 AF680 | 200 µg/ml | $364.00 | |||
AGL Antibody (B-11) Alexa Fluor® 790 | sc-518176 AF790 | 200 µg/ml | $364.00 |