GLA polyclonal Antibody | BS7679

GLA polyclonal Antibody | BS7679 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human

Application: WB

Application Range: WB 1:500 - 1:2000

Background: α-galactosidase A (α-gal A) functions as a lysosomal hydrolase. α-gal A forms an active homodimer that acts upon a glycolipid substrate, globotriaosylceramide (Gb3) . The gene encoding α-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, α-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 α-gal A effectively treats the symptoms of Fabry disease.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: GLA polyclonal Antibody detects endogenous levels of GLA protein.

Molecular Weight: ~ 49 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: 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.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: