ArtNr |
BS7679-50 |
Hersteller |
Bioworld Technology, Inc
|
Menge |
50ul |
Kategorie |
|
Typ |
Antibody Polyclonal |
Applikationen |
WB, IHC |
Specific against |
Human, Mouse, Rat |
Host |
Rabbit |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
AGAL_HUMAN, Agalsidase alfa, Alpha D galactosidase A, Alpha D galactoside galactohydrolase 1, Alpha D galactoside galactohydrolase, Alpha gal A, Alpha galactosidase A, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Alpha-galactosi |
Similar products |
GLA |
Lieferbar |
|
Swiss-Prot |
P06280 |
Application all |
WB: 1:500ca.1:2000 IHC: 1:50ca.1:200 |
Background |
alpha-galactosidase A (alpha-gal A) functions as a lysosomal hydrolase. alpha-gal A forms an active homodimer that acts upon a glycolipid substrate, globotriaosylceramide (Gb3). The gene encoding alpha-gal A maps to chromosome Xq22. Inherited mutations in this gene cause an X-linked recessive glycolipid storage disorder known as Fabry's disease. In Fabry patients, alpha-gal A deficiencies lead to an accumulation of Gb3 in the body. The numerous clinical manifestations of the disease include renal and cardiac impairment, severe pain in the extremities and cutaneous lesions known as angiokeratomas. Enzyme replacement therapy using recombinant alpha-gal A effectively treats the symptoms of Fabry disease. |
Product |
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol. |
Purification and Purity |
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE). |
Storage and Stability |
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles. |
Specificity |
GLA pAb detects endogenous levels of GLA protein. |
BiowMW |
ca. 49 kDa |
Note |
For research use only, not for use in diagnostic procedure. |
Alternative Name |
AGAL_HUMAN, Agalsidase alfa, Alpha D galactosidase A, Alpha D galactoside galactohydrolase 1, Alpha D galactoside galactohydrolase, Alpha gal A, Alpha galactosidase A, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Alpha-galactosidase A, GALA, Galactosidase, alpha, GLA, GLA protein, Melibiase, |
Immunogen |
Recombinant full length Human GLA. |
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Alle Produkte sind nur für Forschungszwecke bestimmt. Nicht für den menschlichen, tierärztlichen oder therapeutischen Gebrauch.