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alpha-Galactosidase A / GLA Antibody, Rabbit PAb, Antigen Affinity Purified

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Mouse GLA Antibody Product Information
Immunogen:Recombinant Mouse alpha-Galactosidase A protein (Catalog#50964-M08H)
Clone ID:
Ig Type:Rabbit IgG
Formulation:0.2 μm filtered solution in PBS
Preparation:Produced in rabbits immunized with purified, recombinant Mouse alpha-Galactosidase A (rh alpha-Galactosidase A; Catalog#50964-M08H; Q8BGZ6; Met1-Arg421). alpha-Galactosidase A specific IgG was purified by Mouse alpha-Galactosidase A affinity chromatography.
Mouse GLA Antibody Usage Guide
Specificity:Mouse alpha-Galactosidase A

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 Mouse alpha-Galactosidase A. The detection limit for Mouse alpha-Galactosidase A is 0.00975 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 -80℃. 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.
Other GLA Antibody Products
alpha-Galactosidase A 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.

Mouse alpha-Galactosidase A References
  • Koide 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.
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