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.

Specificity:

GLA polyclonal antibody detects endogenous levels of GLA protein.

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;

Species reactivity:

Human

Host:

Rabbit

Ig type:

Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Immunogen:

Recombinant full length Human GLA.

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).

Molecular weight:

~ 49 kDa

Applications:

WB

Usage:

WB 1:500 - 1:2000

Storage:

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

Precautions:

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

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