catalog number :
MBS756272
products type :
ELISA Kit
products full name :
Human Gamma secretase ELISA Kit
products short name :
Gamma secretase
other names :
glucan (1,4-alpha-), branching enzyme 1 (glycogen branching enzyme, Andersen disease, glycogen storage disease type IV), isoform CRA_b; 1,4-alpha-glucan-branching enzyme; 1,4-alpha-glucan-branching enzyme; brancher enzyme; glycogen branching enzyme; glycogen-branching enzyme; amylo-(1,4 to 1,6) transglucosidase; amylo-(1,4 to 1,6) transglycosylase; glucan (1,4-alpha-), branching enzyme 1; Brancher enzyme; Glycogen-branching enzyme
products gene name :
Gamma secretase
other gene names :
GBE1; GBE1; GBE; APBD; GSD4
uniprot entry name :
GLGB_HUMAN
specificity :
This assay has high sensitivity and excellent specificity for detection of GSEC. No significant cross-reactivity or interference between GSEC and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between GSEC and all the analogues, therefore, cross reaction may still exist in some cases.
storage stability :
Store all reagents at 2-8 degree C.
other info1 :
Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 0.1 ng/mL.
other info2 :
Intended Uses: This GSEC ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human GSEC. This ELISA kit for research use only, not for therapeutic or diagnostic applications!
products description :
Principle of the assay: GSEC ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-GSEC antibody and an GSEC-HRP conjugate. The assay sample and buffer are incubated together with GSEC-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the GSEC concentration since GSEC from samples and GSEC-HRP conjugate compete for the anti-GSEC antibody binding site. Since the number of sites is limited, as more sites are occupied by GSEC from the sample, fewer sites are left to bind GSEC-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GSEC concentration in each sample is interpolated from this standard curve.
ncbi acc num :
EAW68880.1
ncbi mol weight :
80,474 Da
ncbi pathways :
Disease Pathway (530764); Glucose Metabolism Pathway (106199); Glycogen Metabolism Pathway (198856); Glycogen Storage Diseases Pathway (980468); Glycogen Synthesis Pathway (905990); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Carbohydrates Pathway (106196); Myoclonic Epilepsy Of Lafora Pathway (980469); Starch And Sucrose Metabolism Pathway (82974)
ncbi summary :
The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008]
uniprot summary :
GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen s disease). Protein type: Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.18; Transferase. Chromosomal Location of Human Ortholog: 3p12.3. Cellular Component: cytosol. Molecular Function: cation binding; 1,4-alpha-glucan branching enzyme activity; hydrolase activity, hydrolyzing O-glycosyl compounds. Biological Process: glycogen metabolic process; generation of precursor metabolites and energy; glycogen biosynthetic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis. Disease: Polyglucosan Body Disease, Adult Form; Glycogen Storage Disease Iv