Glycogen debranching enzyme; Glycogen debrancher; AGL; GDE

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

Store at 4 degree C

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Small volumes of AGL elisa kit 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.

MBS9427551 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glycogen debranching enzyme (AGL) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing AGL. The ELISA analytical biochemical technique of the MBS9427551 kit is based on AGL antibody-AGL antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect AGL antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, AGL. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

172,696 Da

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

glycogen debranching enzyme

Glycogen debranching enzyme

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

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; 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; isoamylase complex

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

Biological Process: glycogen catabolic process; neutrophil degranulation

Disease: Glycogen Storage Disease Iii

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