ELISA/assay

Human alpha-Galactosidase ELISA Kit

MBS743980

48-Strip-Wells-(Competitive)

440 USD

ELISA Kit

Human alpha-Galactosidase ELISA Kit

[alpha-Galactosidase]

[Human a-Galactosidase ELISA Kit]

[alpha-galactosidase, partial; Alpha-galactosidase A; alpha-galactosidase A; melibiase; alpha-gal A; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; galactosidase, alpha; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase]

[alphaGAL]

[aGAL]

[GLA; GLA; GALA]

AGAL_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of GLYC. No significant cross-reactivity or interference between GLYC and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between GLYC and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C

Typical Testing Data/Standard Curve (for reference only)

Samples: Serum, Plasma, Cell Culture Supernatants, Body Fluid And Tissue Homogenate. Assay Type: Quantitative Competitive. Sensitivity: 1.0 pg/mL.

Cardiovascular

Intended Uses: This GLYC ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human GLYC. This ELISA kit for research use only, not for therapeutic or diagnostic applications!. Principle of the Assay: GLYC ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-GLYC antibody and an GLYC-HRP conjugate. The assay sample and buffer are incubated together with GLYC-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 GLYC concentration since GLYC from samples and GLYC-HRP conjugate compete for the anti-GLYC antibody binding site. Since the number of sites is limited, as more sites are occupied by GLYC from the sample, fewer sites are left to bind GLYC-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GLYC concentration in each sample is interpolated from this standard curve.

553299

AAA52514.1

P06280

48,767 Da

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Glycerolipid Metabolism Pathway (82986); Glycerolipid Metabolism Pathway (361); Glycosphingolipid Biosynthesis - Globo Series Pathway (82996); Glycosphingolipid Biosynthesis - Globo Series Pathway (371); Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135)

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]

GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family. Protein type: Lipid Metabolism - sphingolipid; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; EC 3.2.1.22; Lipid Metabolism - glycerolipid; Carbohydrate Metabolism - galactose; Hydrolase. Chromosomal Location of Human Ortholog: Xq22. Cellular Component: Golgi apparatus; lysosomal lumen; lysosome; cytoplasm; extracellular region. Molecular Function: protein binding; protein homodimerization activity; hydrolase activity; alpha-galactosidase activity; galactoside binding; catalytic activity; receptor binding. Biological Process: sphingolipid metabolic process; glycoside catabolic process; negative regulation of nitric-oxide synthase activity; negative regulation of nitric oxide biosynthetic process; glycosphingolipid catabolic process; glycosylceramide catabolic process; glycosphingolipid metabolic process; oligosaccharide metabolic process. Disease: Fabry Disease

48-Strip-Wells-(Competitive)

440 USD

48-Strip-Wells-(Sandwich)

440

96-Strip-Wells-(Competitive)

640

96-Strip-Wells-(Sandwich)

640

5x96-Strip-Wells-(Competitive)

2895

5x96-Strip-Wells-(Sandwich)

2895

10x96-Strip-Wells-(Competitive)

5415

10x96-Strip-Wells-(Sandwich)

5415