Alpha-galactosidase A, Polyclonal Antibody

Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; Agalsidase

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of Alpha-galactosidase A protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-GLA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to Alpha-galactosidase A

Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)

WB: 1/500 - 1/2000; IF/ICC: 1/50 - 1/100

Western blot analysis of Alpha-galactosidase A expression in SW620 (A), Hela (B) whole cell lysates.

Immunofluorescent analysis of Alpha-galactosidase A staining in Hela cells. Formalin-fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 5-10 minutes and blocked with 3% BSA-PBS for 30 minutes at room temperature. Cells were probed with the primary antibody in 3% BSA-PBS and incubated overnight at 4 °C in a humidified chamber. Cells were washed with PBST and incubated with a DyLight 594-conjugated secondary antibody (red) in PBS at room temperature in the dark.

48,767 Da

galactosidase alpha

alpha-galactosidase A

Alpha-galactosidase A

AGAL_HUMAN

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

Disease: Fabry Disease

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