Human Glycogen Debranching Enzyme (AGL) ELISA Kit | AGL elisa kit

Human Glycogen Debranching Enzyme (AGL) ELISA Kit

Cat. Num. AAA2122186

• Gene Names: AGL; GDE
• Reactivity: Human
• Synonyms: Glycogen Debranching Enzyme (AGL); Human Glycogen Debranching Enzyme (AGL) ELISA Kit; GDE; Amylo-1; 6-Glucosidase; 4-Alpha-Glucanotransferase; Glycogen debrancher; 4-alpha-glucanotransferase; Oligo-1; 4-1; 4-glucantransferase; Dextrin 6-alpha-D-glucosidase; AGL elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of Glycogen Debranching Enzyme (AGL).; No significant cross-reactivity or interference between Glycogen Debranching Enzyme (AGL) and analogues was observed.
• Sequence Length: 1532

• Assay Type: Double-antibody Sandwich
• Sample: Tissue Homogenates, Cell Lysates and Other Biological Fluids
• Dectection Range: 0.156-10ng/mL
• Sensitivity: The minimum detectable dose of this kit is typically less than 0.057ng/mL
• Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Glycogen Debranching Enzyme (AGL) were tested 20 times on one plate, respectively.; Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Glycogen Debranching Enzyme (AGL) were tested on 3 different plates, 8 replicates in each plate.; CV(%) = SD/meanX100; Intra-Assay: CV<10%; Inter-Assay: CV<12%
• Preparation and Storage: The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition.; To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for AGL elisa kit

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Glycogen Debranching Enzyme (AGL). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Glycogen Debranching Enzyme (AGL). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Glycogen Debranching Enzyme (AGL), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of Glycogen Debranching Enzyme (AGL) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

Product Categories/Family for AGL elisa kit

Enzyme & Kinase

NCBI and Uniprot Product Information

• NCBI GI #: 116734847
• NCBI GeneID: 178
• NCBI Accession #: NP_000019.2
• NCBI GenBank Nucleotide #: NP_000019.2
• UniProt Accession #: P35573
• 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