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Product Details
BS-7593R
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
KLH conjugated synthetic peptide derived from human Galactosidase alpha, amino acids 101-200.
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein A
Storage buffer
0.01M TBS, pH 7.4, with 1% BSA, 50% glycerol
Contains
0.02% ProClin 300
Storage conditions
-20°C
Shipping conditions
Ambient (domestic); Wet ice (international)
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: agalsidase alfa; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; alpha-D-galactoside galactohydrolase 1; alpha-gal A; Alpha-galactosidase A; galactosidase, alpha; Galactosylgalactosylglucosylceramidase GLA; Melibiase
Gene Aliases: Ags; GALA; GLA
UniProt ID: (Human) P06280
Entrez Gene ID: (Human) 2717, (Mouse) 11605
catalytic activity
alpha-galactosidase activity
receptor binding
protein binding
hydrolase activity
galactoside binding
protein homodimerization activity
raffinose alpha-galactosidase activity
hydrolase activity, hydrolyzing O-glycosyl compounds
hydrolase activity, acting on glycosyl bonds
galactosidase
glycosphingolipid metabolic process
oligosaccharide metabolic process
negative regulation of nitric oxide biosynthetic process
glycosylceramide catabolic process
glycosphingolipid catabolic process
negative regulation of nitric-oxide synthase activity
carbohydrate metabolic process
metabolic process
glycoside catabolic process
bone mineralization
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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