Galactosidase alpha (GLA) Polyclonal Antibody-BS77041

Galactosidase alpha (GLA) Polyclonal Antibody

Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Sizes: 50µl, 100µl

Catalogue Numbers: BS77041-50, BS77041-100

Swiss-Prot: P06280

Host: Rabbit

Galactosidase alpha (GLA) Polyclonal Antibody-BS77041

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Reactivity: Human, Mouse

Applications: WB, IF/ICC

All Applications: WB,1:500 - 1:2000 | IF/ICC,1:50 - 1:100

Background: 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.

Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Specificity: Unmodification

Bioworld Molecular Weight: 49kDa

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Western blot analysis of extracts of various cell lines, using Galactosidase alpha ) antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% non-fat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 30s., Immunofluorescence analysis of HeLa cells using Galactosidase alpha ) antibody. Blue: DAPI for nuclear staining.

Alternative Name: GLA; GALA

Immunogen: Recombinant fusion protein of human Galactosidase alpha (Galactosidase alpha (GLA))(NP_000160.1).

Modification: Unmodified

Conjugate: Unconjugated