alpha-Galactosidase A / GLA Antibody

alpha-Galactosidase A / GLA Antibody Datasheet

alpha-Galactosidase A / GLA Antibody Product Information

• Immunogen: Recombinant Human alpha-Galactosidase A / GLA protein (Catalog#12078-H08H)
• Antibody Type: Mouse Monoclonal Antibody ( Mouse mAb Service Platform )
• Clone ID: 2H7G10
• Ig Type: Mouse IgG2b
• Formulation: 0.2 μm filtered solution in PBS with 5% trehalose
• Preparation: This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; Catalog#12078-H08H; NP_000940.1; Met 1-Leu 429). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.

alpha-Galactosidase A / GLA Antibody Usage Guide

• Specificity: Human alpha-Galactosidase A / GLA
• No cross-reactivity in ELISA with

  Human S100A8

  Human S100A1

  Human S100P

  Human cell lysate (293 cell line)

• Western blot: This antibody can be used at 1-2 μg/mL with the appropriate secondary reagents to detect Human GLA in WB. Using a DAB detection system, the detection limit for Human GLA is approximately 2 ng/lane under non-reducing conditions and 1 ng/lane under reducing conditions.
• Direct ELISA: This antibody can be used at 0.5-1 μg/mL with the appropriate secondary reagents to detect Human GLA. The detection limit for Human GLA is approximately 0.039 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.

alpha-Galactosidase A / GLA Antibody 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

1. Koide T.et al., 1990, FEBS Lett. 259:353-356.
2. Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
3. Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
4. Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.