AN006790L
GLA Alpha/Galactosidase Alpha Polyclonal Antibody
Größe
£120.60
War £134.00
- SKU:
- AN006790L
- Zusätzliche Namen:
- GLA,GALA,Alpha-D-Galactoside,Galactohydrolase,galactosidase alpha,GLAL,Melibiase,Agalsidase,Alpha-galactosidase A,Alpha-D-galactosidase A,Alpha-D-galactoside galactohydrolase,Galactosylgalactosylglucosylceramidase GLA
- Anwendung:
- IHC, WB
- Konzentration:
- 1 mg/ml
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Elabscience
- Host:
- Rabbit
- Reaktivitäten:
- Human
- Buffer:
- PBS with 0.05% Proclin300, 1% protective protein and 50% glycerol, pH7.4
- Immunogen:
- Recombinant Human GLA Alpha/Galactosidase Alpha protein expressed by Mammalian
- Uniprot:
- P06280
- Synonyme:
- 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
- Weitere 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.
- Versandbedingungen:
- Blue Ice