Bovine alpha-Galactosyl ELISA Kit | MyBioSource MBS006878 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Bovine alpha-Galactosyl ELISA Kit

catalog :

MBS006878

quantity :

48-Strip-Wells

price :

470 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS006878

products type :

ELISA Kit

products full name :

Bovine alpha-Galactosyl ELISA Kit

products short name :

[alpha-Galactosyl]

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]

products gene name :

[alpha-GAL]

other gene names :

[GLA; GLA; GALA]

uniprot entry name :

AGAL_HUMAN

reactivity :

Bovine

sequence length :

429

specificity :

No significant cross-reactivity or interference between this analyte and analogues is observed.

storage stability :

Store all reagents at 2-8 degree C

other info1 :

Samples: Hamster Body Fluids, Tissue Homogenates, Secretions Or Feces Samples. Assay Type: Quantitative Sandwich. Detection Range: 0.625 ng/ml-20 ng/ml. Sensitivity: 0.1 ng/ml.

other info2 :

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].

products description :

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of AGA (hereafter termed "analyte") in undiluted original Hamster body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

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: EC 3.2.1.22; Lipid Metabolism - glycerolipid; Carbohydrate Metabolism - galactose; Lipid Metabolism - sphingolipid; Hydrolase; Glycan Metabolism - glycosphingolipid biosynthesis - globo series. 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 :

470 USD

size2 :

96-Strip-Wells

price2 :

680

size3 :

5x96-Strip-Wells

price3 :

3100

size4 :

10x96-Strip-Wells

price4 :

6095

more info or order :

MyBioSource product webpage