BS7679

antibody from Bioworld Technology, Inc
Targeting: GLA GALA
Provider product page for BS7679
Western blot
Immunocytochemistry
Immunohistochemistry

Antibody data

Product number
BS7679 - Provider product page
Provider
Bioworld Technology, Inc
Product name
GLA polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human GLA.
Description
Ï«-galactosidase A (Ï«-gal A) functions as a lysosomal hydrolase. Ï«-gal A forms an active homodimer that acts upon a glycolipid substrate, globotriaosylceramide (Gb3). The gene encoding Ï«-gal A maps to chromosome Xq22. Inherited mutations in this gene cause an X-linked recessive glycolipid storage disorder known as Fabry£ªs disease. In Fabry patients, Ï«-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 Ï«-gal A effectively treats the symptoms of Fabry disease.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
GLA protein structure - BS7679 shown in red.