Glycogen debranching enzyme; Glycogen debrancher; 4-alpha-glucanotransferase; Oligo-1;4-1;4-glucantransferase; Amylo-alpha-1;6-glucosidase; Amylo-1;6-glucosidase; Dextrin 6-alpha-D-glucosidase; AGL; GDE

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

Polyclonal

Ig

Rabbit

This AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the N-terminal region of human AGL.

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-AGL antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: AGL is a 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 the AGL 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.

Background: 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.

Primary Antibodies; Cancer; Signal Transduction

Western Blot (WB), Immunofluorescence (IF), ELISA (EIA)

WB:~~1:1000
IF:~~1:10~50

Anti-AGL Antibody (M15) at 1:1000 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.

Expression of myc-GS causes wild type but not the ?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 ?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.

174764 Da

amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase

glycogen debranching enzyme

Glycogen debranching enzyme

GDE; Amylo-1,6-glucosidase??[Similar Products]

GDE_HUMAN

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]

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; Hydrolase; EC 3.2.1.33; Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.25; Transferase

Chromosomal Location of Human Ortholog: 1p21

Cellular Component: cytoplasm; cytosol; inclusion body; isoamylase complex; nucleus; sarcoplasmic reticulum

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

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

Disease: Glycogen Storage Disease Iii

Horinishi, A., et al., J. Hum. Genet. 47 (2):55-59 (2002). Shen, J., et al., Hum. Mutat. 9 (1):37-40 (1997). Bao, Y., et al., Genomics 38 (2):155-165 (1996). Shen, J., et al., J. Clin. Invest. 98 (2):352-357 (1996). Yang, B.Z., et al., J. Biol. Chem. 267 (13):9294-9299 (1992).

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