Recombinant alpha-Galactosidase A/GLA Monoclonal Antibody

Size

£170.10

Was £189.00

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:: WB
Concentration:: 1 mg/ml
Purification:: Protein A Purified
Storage Conditions:: -20[o]C/-70[o]C Avoid freeze/thaw cycles., 2-8[o]C Avoid freeze/thaw cycles.
Supplier:: Elabscience
Host:: Rabbit
Reactivities:: Human
Buffer:: PBS
Immunogen:: Recombinant Human alpha-Galactosidase A/GLA protein
Clone:: B338
Uniprot:: P06280
Synonyms:: agalsidase alfa;alpha-D-galactosidase A;Alpha-D-galactoside galactohydrolase;alpha-D-galactoside galactohydrolase 1;alpha-gal A;alpha-galactosidase A;GALA;galactosylgalactosylglucosylceramidase GLA;melibiase
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 .