AGL; GDE; glycogen debranching enzyme

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

Polyclonal

IgG

Rabbit

Affinity purification

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Store at -20°C. Avoid 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.

This gene encodes the glycogen debrancher enzyme which is involved in glycogendegradation. This enzyme has two independent catalytic activities which occur at different siteson 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 ofenzymatic and clinical variability occurs which may be due to tissue-specific alternativesplicing. Alternatively spliced transcripts encoding different isoforms have been described.

Polyclonal

Western Blot (WB)

WB: 1:500 - 1:2000

Western blot analysis of extracts of various cell lines, using AGL antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 90s.

Calculated MW: 172 kDa/174 kDa
Observed MW: 174 kDa

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

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: carbohydrate metabolic process; glucose metabolic process; glycogen biosynthetic process; glycogen catabolic process; response to glucocorticoid stimulus; response to nutrient

Disease: Glycogen Storage Disease Iii

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