Human alpha-galactosidase, alphaGAL ELISA Kit | MyBioSource MBS702399 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human alpha-galactosidase, alphaGAL ELISA Kit

catalog :

MBS702399

quantity :

48-Strip-Wells

price :

565 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS702399

products type :

ELISA Kit

products full name :

Human alpha-galactosidase, alphaGAL ELISA Kit

products short name :

galactosidase, alpha

products name syn :

Human alpha-galactosidase; alphaGAL ELISA Kit; GALA; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; alpha-gal A; alpha-galactosidase A; melibiase; galactosidase; alpha

other names :

alpha-galactosidase A; 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; INN: Agalsidase

products gene name :

GLA

other gene names :

GLA; GLA; GALA

uniprot entry name :

AGAL_HUMAN

reactivity :

Human

sequence length :

429

specificity :

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

storage stability :

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

other info1 :

Samples: Serum, plasma, tissue homogenates. Assay Type: Sandwich. Detection Range: 7.8 pg/ml -500 pg/ml. Sensitivity: 1.95 pg/ml.

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

products description :

Principle of the Assay This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for alphaGAL has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any alphaGAL present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for alphaGAL is added to the wells. After washing, avidin 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 alphaGAL bound in the initial step. The color development is stopped and the intensity of the color is measured.

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 (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135)

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: Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Carbohydrate Metabolism - galactose; Lipid Metabolism - glycerolipid; EC 3.2.1.22; Lipid Metabolism - sphingolipid. 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; negative regulation of nitric-oxide synthase activity; glycoside catabolic process; negative regulation of nitric oxide biosynthetic process; glycosphingolipid catabolic process; glycosylceramide catabolic process; glycosphingolipid metabolic process; oligosaccharide metabolic process. Disease: Fabry Disease

size1 :

48-Strip-Wells

price1 :

565 USD

size2 :

96-Strip-Wells

price2 :

810

size3 :

5x96-Strip-Wells

price3 :

2750

size4 :

10x96-Strip-Wells

price4 :

5150

more info or order :

MyBioSource product webpage