Human Glycogen debranching enzyme (AGL) ELISA Kit | MyBioSource MBS281089 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Glycogen debranching enzyme (AGL) ELISA Kit

catalog :

MBS281089

quantity :

48-Strip-Wells

price :

405 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS281089

products type :

ELISA Kit

products full name :

Human Glycogen debranching enzyme (AGL) ELISA Kit

products short name :

[Glycogen debranching enzyme]

other names :

[glycogen debranching enzyme isoform 1; Glycogen debranching enzyme; glycogen debranching enzyme; amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase; Glycogen debrancherIncluding the following 2 domains:4-alpha-glucanotransferase (EC:2.4.1.25)Alternative name(s):Oligo-1,4-1,4-glucantransferase]

products gene name :

[AGL]

other gene names :

[AGL; AGL; GDE; GDE; Amylo-1,6-glucosidase]

reactivity :

Human

sequence length :

711

specificity :

This assay has high sensitivity and excellent specificity for detection of Human RGP. No significant cross-reactivity or interference between Human RGP and analogues was observed.

storage stability :

Store at 2-8 degree C.

other info1 :

Samples: Serum, Plasma, Saliva And Other Biological Fluids. Assay Type: Quantitative Sandwich. Detection Range: 31.25 pg/mL-2000 pg/mL. Sensitivity: 10.4 pg/mL (mean of 6 independent assays).

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay) Three samples of known concentration were tested twenty times on one plate to assess intra-assay precision. Intra-Assay: CV<8%. Inter-assay Precision: Inter-assay Precision (Precision between assays) Three samples of known concentration were tested in forty separate assays to assess inter-assay precision. CV (%) = SD/meanX100. Inter-Assay: CV<12%

products description :

Intended Uses: For the quantitative detection of Human Gingipain R (RGP) concentration in serum, plasma, saliva and other biological fluids. Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate RGP in Human serum, plasma, saliva. An antibody specific for RGP has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any RGP present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for RGP is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of RGP bound in the initial step. The color development is stopped and the intensity of the color is measured.

ncbi gi num :

116734847

ncbi acc num :

NP_000019.2

ncbi gb acc num :

NM_000028.2

uniprot acc num :

P35573

ncbi mol weight :

172,696 Da

ncbi pathways :

Glucose Metabolism Pathway (1269959); Glycogen Metabolism Pathway (198856); Glycogen Breakdown (glycogenolysis) Pathway (1269963); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Carbohydrates Pathway (1269957); Starch And Sucrose Metabolism Pathway (82974); Starch And Sucrose Metabolism Pathway (344)

ncbi summary :

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 summary :

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

size1 :

48-Strip-Wells

price1 :

405 USD

size2 :

96-Strip-Wells

price2 :

675

size3 :

5x96-Strip-Wells

price3 :

3080

size4 :

10x96-Strip-Wells

price4 :

5805

more info or order :

MyBioSource product webpage