Antibody data
- Antibody Data
- Antigen structure
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- 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.
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