Human Alpha-galactosidase A ELISA Kit | MyBioSource MBS2881234 product information

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product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Alpha-galactosidase A ELISA Kit

catalog :

MBS2881234

quantity :

48-Strip-Wells

price :

355 USD

product information

catalog number :

MBS2881234

products type :

ELISA Kit

products full name :

Human Alpha-galactosidase A ELISA Kit

products short name :

[Alpha-galactosidase A]

products name syn :

[Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; GLA; 3.2.1.22]

other names :

[alpha-galactosidase A; Alpha-galactosidase A; alpha-galactosidase A; galactosidase alpha; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; INN: Agalsidase]

products gene name :

[GLA]

other gene names :

[GLA; GLA; GALA]

uniprot entry name :

AGAL_HUMAN

reactivity :

Human

sequence length :

429

specificity :

Recombinant and natural Human Melanoma-associated antigen 4

storage stability :

For long term storage, please store the entire kit at -20 degree C.

image1 heading :

Typical Testing Data/Standard Curve (for reference only)

other info1 :

Samples: Serum, Plasma, Tissue Homogenates, Cell Culture Supernates Or Other Biological Fluids. Detection Range: 0.78-50 ng/mL. Sensitivity: <0.395 ng/mL

other info2 :

Intra-assay Precision: Intra-Assay CV: <=4.7%. Inter-assay Precision: Inter-Assay CV: <=8.5%

products description :

Intended Uses: This immunoassay kit allows for the in vitro quantitative determination of target antigen concentrations in serum, plasma, tissue homogenates, cell culture supernates or other biological fluids. Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to target antigen. Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody preparation specific for target antigen and then avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then a TMB substrate solution is added to each well. Only those wells that contain target antigen, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The concentration of target antigen in the samples is then determined by comparing the O.D. of the samples to the standard curve.

ncbi gi num :

4504009

ncbi acc num :

NP_000160.1

ncbi gb acc num :

NM_000169.2

uniprot acc num :

P06280

ncbi mol weight :

48,767 Da

ncbi pathways :

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 (1270099); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (1269956)

ncbi summary :

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]

uniprot summary :

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; Carbohydrate Metabolism - galactose; Lipid Metabolism - glycerolipid; Hydrolase; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; EC 3.2.1.22. Chromosomal Location of Human Ortholog: Xq22. Cellular Component: cytoplasm; extracellular region; Golgi apparatus; lysosomal lumen; lysosome. Molecular Function: alpha-galactosidase activity; catalytic activity; galactoside binding; hydrolase activity; protein binding; protein homodimerization activity; receptor binding. Biological Process: glycosphingolipid catabolic process; glycosphingolipid metabolic process; glycosylceramide catabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; oligosaccharide metabolic process; sphingolipid metabolic process. Disease: Fabry Disease

size1 :

48-Strip-Wells

price1 :

355 USD

size2 :

96-Strip-Wells

price2 :

475

size3 :

5x96-Strip-Wells

price3 :

2030

size4 :

10x96-Strip-Wells

price4 :

3700