BackgroundThis 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.
DescriptionGLA Recombinant Monoclonal Antibody [30H11]. Unconjugated. Raised in: HEK293F Cell.
FormulationBuffer: Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
SpecificityHuman
IsotypeRabbit IgG
Uniprot IDP06280
PurificationAffinity Chromatography
ImmunogenA synthesized peptide derived from Human GLA
StorageUpon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Alternative NamesAlpha-galactosidase A (EC 3.2.1.22) (Alpha-D-galactosidase A) (Alpha-D-galactoside galactohydrolase) (Melibiase) (Agalsidase), GLA
ApplicationELISA, IHC, FC; Recommended dilution: IHC:1:50-1:200, FC:1:50-1:200