Anti-GLA Antibody

Cat: 12078-MM07
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Anti-GLA Antibody (Mouse Monoclonal antibody) General Information
Product name
Anti-GLA Antibody
Validated applications
ELISA
Species reactivity
Reacts with: Human
Specificity
Human GLA
No cross-reactivity in ELISA with Human S100A8 Human S100A1 Human S100P Human cell lysate (293 cell line)
Immunogen
Recombinant Human alpha-Galactosidase A / GLA protein (Catalog#12078-H08H)
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; AAP36507.1; Met 1-Leu 429). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
Source
Monoclonal Mouse IgG2b Clone #2H7G10
Purification
Protein A
Formulation
0.2 μm filtered solution in PBS
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
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.
Anti-GLA Antibody (Mouse Monoclonal antibody) Validated Applications
Application Dilution Notes
ELISA 1:1000-1:2000  

**********Please Note: Optimal concentrations/dilutions should be determined by the end user.**********

Anti-GLA Antibody Alternative Names
Anti-GALA Antibody
GLA Background Information

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.

Full Name
galactosidase, alpha
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.
  • Product Description Host Clonality Application Catalog# (PDF)
    Anti-GLA Antibody Rabbit Polyclonal ELISA 50964-RP01
    Anti-GLA Antibody Rabbit Polyclonal ELISA 50964-T16
    Anti-GLA Antibody Rabbit Polyclonal ELISA,IP 12078-T20
    Anti-GLA Antibody Rabbit Polyclonal ELISA 12078-T16
    Anti-GLA Antibody Mouse Monoclonal ELISA,ELISA(Cap) 12078-MM08
    Anti-GLA Antibody Rabbit Polyclonal WB,ELISA,IP 12078-T52
    Anti-GLA Antibody Mouse Monoclonal ELISA(Det) 12078-MM05
    Anti-GLA Antibody Rabbit Monoclonal WB,ELISA,IP 12078-R001
    Anti-GLA Antibody Rabbit Monoclonal WB 12078-R201
    Anti-GLA Antibody Rabbit Polyclonal ELISA 12078-RP01
    Anti-GLA Antibody Rabbit Polyclonal WB,ELISA,IHC-P,IP 12078-RP02
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