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Antibodies

Recombinant alpha-Galactosidase A/GLA Monoclonal Antibody

Product Sizes
20 ul
£189.00
AN300412P-20UL
100 ul
£402.00
AN300412P-100UL
About this Product
SKU:
AN300412P
Additional Names:
GLA,GALA,Alpha-D-Galactoside,Galactohydrolase,galactosidase alpha,GLAL,Melibiase,Agalsidase,Alpha-galactosidase A,Alpha-D-galactosidase A,Alpha-D-galactoside galactohydrolase,Galactosylgalactosylglucosylceramidase GLA
Application:
Western Blot
Buffer:
PBS
translate.label.attr.clone:
B338
Clonality:
Monoclonal
Concentration:
1 mg/ml
Extra Details:
alpha -Galactosidase A is a homodimeric glycoprotein that can release terminal alpha -galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose . It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1-4Gal beta 1-4Glc beta -ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes .
Host:
Rabbit
Immunogen:
Recombinant Human alpha-Galactosidase A/GLA protein
Isotype:
IgG
Purification:
Protein A Purified
Reactivities:
Human
Shipping Conditions:
Blue Ice
Storage Conditions:
-20[o]C/-70[o]C Avoid freeze/thaw cycles., 2-8[o]C Avoid freeze/thaw cycles.
Supplier:
Elabscience
Type:
Antibody: Monoclonal Antibody