Rabbit anti-Human AGL Polyclonal Antibody | anti-AGL antibody

AGL, CT (AGL, GDE, Glycogen debranching enzyme, Glycogen debrancher, 4-alpha-glucanotransferase, Oligo-1,4-1,4-glucantransferase, Amylo-alpha-1,6-glucosidase, Dextrin 6-alpha-D-glucosidase) (AP)

Cat. Num. AAA6272234

• Gene Names: AGL; GDE
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: AGL; Polyclonal Antibody; CT (AGL; GDE; Glycogen debranching enzyme; Glycogen debrancher; 4-alpha-glucanotransferase; Oligo-1; 4-1; 4-glucantransferase; Amylo-alpha-1; 6-glucosidase; Dextrin 6-alpha-D-glucosidase) (AP); Dextrin 6-alpha-D-glucosidase; anti-AGL antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-AGL antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1487~1516 amino acids from the C-terminal region of human AGL.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot using anti-AGL (C-term) antibody (AP2402c) 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.)

Testing Data

(Expression of myc-GS causes wild type but not the Ã"CBD 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 Ã"CBD. 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)

(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).)

Product Categories/Family for anti-AGL antibody

Antibodies; Enzymes

References

Horinishi, A., et al., J. Hum. Genet. 47(2):55-59 (2002). 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 GI #: 116734847
• NCBI GeneID: 178
• NCBI Accession #: NP_000019.2
• NCBI GenBank Nucleotide #: NM_000028.2
• UniProt Accession #: P35573; P78354; P78544; Q59H92; Q6AZ90; Q9UF08; A6NCX7; A6NEK2; D3DT51
• Molecular Weight: 172,696 Da
• NCBI Official Full Name: glycogen debranching enzyme isoform 1
• NCBI Official Synonym Full Names: amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
• NCBI Official Symbol: AGL
• NCBI Official Synonym Symbols: GDE
• NCBI Protein Information: glycogen debranching enzyme
• UniProt Protein Name: Glycogen debranching enzyme
• UniProt Gene Name: AGL
• UniProt Synonym Gene Names: GDE; Amylo-1,6-glucosidase

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: Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.25; EC 3.2.1.33; Hydrolase; Transferase; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 1p21.2

Cellular Component: cytoplasm; cytosol; extracellular region; ficolin-1-rich granule lumen; inclusion body; isoamylase complex; nucleus; sarcoplasmic reticulum; secretory granule lumen

Molecular Function: 4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; glycogen debranching enzyme activity; polysaccharide binding; polyubiquitin modification-dependent protein binding; protein binding

Biological Process: glycogen biosynthetic process; glycogen catabolic process; neutrophil degranulation; response to glucocorticoid stimulus; response to nutrient

Disease: Glycogen Storage Disease Iii