Western Blot
( Anti-AGL Antibody (C-term) at 1:8000 dilution + human skeletal muscle lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 175 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
Rabbit anti-Human AGL Polyclonal Antibody | anti-AGL antibody
AGL Antibody (C-term)
IF~~1:10~50
Western Blot
( Anti-AGL Antibody (C-term) at 1:8000 dilution + human skeletal muscle lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 175 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
Western Blot
( Anti-AGL Antibody (C-term) at 1:8000 dilution + human skeletal muscle lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 175 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)
Western Blot (WB)
(Western blot using anti-AGL (C-term) antibody (MBS9217121) at 1:1000 dilution. A total of 20 ug of lysates was loaded for each tissue. Data courtesy of Dr. Alan Cheng, Department of Internal Medicine, Life Sciences Institute, University of Michigan Medical Center, Ann Arbor, Michigan.)
Western Blot (WB)
(Western blot using anti-AGL (C-term) antibody (MBS9217121) at 1:1000 dilution. A total of 20 ug of lysates was loaded for each tissue. Data courtesy of Dr. Alan Cheng, Department of Internal Medicine, Life Sciences Institute, University of Michigan Medical Center, Ann Arbor, Michigan.)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of AGL Antibody (C-term) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red).DAPI was used to stain the cell nuclear (blue).)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of AGL Antibody (C-term) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red).DAPI was used to stain the cell nuclear (blue).)
Immunofluorescence (IF)
(Expression of myc-GS causes wild type but not the ÄCBD mutant of AGL to aggregate around the PAS-stain-positive inclusions. HepG2 cells were transfected with either HA-tagged wild-type AGL (HA-AGL) or HA-AGL ÄCBD. Cells were fixed in formalin and processed for IF using anti-HA (green) and anti-myc (red) antibodies. White arrows indicate colocalization of HA-AGL and myc-GS.)
Immunofluorescence (IF)
(Expression of myc-GS causes wild type but not the ÄCBD mutant of AGL to aggregate around the PAS-stain-positive inclusions. HepG2 cells were transfected with either HA-tagged wild-type AGL (HA-AGL) or HA-AGL ÄCBD. Cells were fixed in formalin and processed for IF using anti-HA (green) and anti-myc (red) antibodies. White arrows indicate colocalization of HA-AGL and myc-GS.)
Bao, Y., et al., Genomics 38(2):155-165 (1996).
Yang, B.Z., et al., J. Biol. Chem. 267(13):9294-9299 (1992).
Yang-Feng, T.L., et al., Genomics 13(4):931-934 (1992).
Bao, Y., et al., Gene 197 (1-2), 389-398 (1997).
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
Uniprot Description
GDE: 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. Defects in AGL are the cause of glycogen storage disease type 3 (GSD3); also known as Forbes disease. GSD3 is a metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. Three GSD3 types are recognized: GSD type 3A patients lack glycogen debrancher enzyme activity in both liver and muscle, while GSD type 3B patients are enzyme- deficient in liver only. In rare cases, selective loss of only 1 of the 2 debranching activities, glucosidase or transferase, results in GSD type 3C or type 3D, respectively. GSD3 is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Belongs to the glycogen debranching enzyme family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Ubiquitin conjugating system; EC 2.4.1.25; Transferase; EC 3.2.1.33; Carbohydrate Metabolism - starch and sucrose; Hydrolase
Chromosomal Location of Human Ortholog: 1p21
Cellular Component: nucleoplasm; sarcoplasmic reticulum; cytoplasm; isoamylase complex; inclusion body; cytosol
Molecular Function: protein binding; glycogen debranching enzyme activity; 4-alpha-glucanotransferase activity; polyubiquitin binding; amylo-alpha-1,6-glucosidase activity; polysaccharide binding
Biological Process: glycogen biosynthetic process; glycogen catabolic process; carbohydrate metabolic process; response to glucocorticoid stimulus; glucose metabolic process; pathogenesis; response to nutrient
Disease: Glycogen Storage Disease Iii
Research Articles on AGL
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Product Notes
The AGL agl (Catalog #AAA9201231) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 1479-1510. The AGL Antibody (C-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's AGL can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA, Immunofluorescence (IF). WB~~1:1000 IF~~1:10~50. Researchers should empirically determine the suitability of the AGL agl 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 "AGL, 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.Item has been added to Shopping Cart
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