ELISA/assay

人类阿尔法半乳糖苷酶, alphaGAL酶联免疫吸附试剂盒

MBS702399

48-Strip-Wells

565美元

酶联免疫吸附试剂盒

人类阿尔法半乳糖苷酶, alphaGAL酶联免疫吸附试剂盒

半乳糖苷酶, 甲

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

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

GLA

GLA;GLA;GALA

AGAL_HUMAN

人类

429

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.

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

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

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.

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.

4504009

NP_000160.1

NM_000169.2

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: 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

48-Strip-Wells

565美元

96-Strip-Wells

810

5x96-Strip-Wells

2750

10x96-Strip-Wells

5150