Quick Order

Text Size:AAA

alpha-Galactosidase A / GLA Antibody, Rabbit MAb

DatasheetSpecific ReferencesReviewsRelated ProductsProtocols
GLAAntibody Product Information
Immunogen:Recombinant Human alpha-Galactosidase A / GLA protein (Catalog#12078-H08H)
Clone ID:001
Ig Type:Rabbit IgG
Concentration:
Formulation:0.2 μm filtered solution in PBS with 5% trehalose
Preparation:This antibody was obtained from a rabbit immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; Catalog#12078-H08H; NP_000940.1; Met 1-Leu 429).
GLAAntibody Usage Guide
Specificity:Human alpha-Galactosidase A / GLA
Application:WB, ELISA, IP

WB: 0.5-2 μg/mL

This antibody can be used at 0.5-2 μg/mL with the appropriate secondary reagents to detect Human GLA in WB.

ELISA: 0.1-0.2 μg/mL

This antibody can be used at 0.1-0.2 μg/mL with the appropriate secondary reagents to detect Human GLA. The detection limit for Human GLA is approximately 0.0195 ng/well.

Storage:This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -70℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.
Background

Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.

References
  • Koide T.et al., 1990, FEBS Lett. 259:353-356.
  • Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
  • Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
  • Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.
  • Related Products
    Product nameProduct name
    Size / Price
    Catalog:12078-R001-50
    List Price: $170.00  (Save $0.00)
    Price:$170.00      [How to order]
    AvailabilityIn Stock
    Images
    • alpha-Galactosidase A / GLA Antibody, Rabbit MAb, Western blot
    • alpha-Galactosidase A / GLA Antibody, Rabbit MAb, Immunoprecipitation
    Recently Viewed Items
      Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"