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Anti-GLA/alpha-Galactosidase A Antibody (2S483)
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Catalog No. TMAY-02227
Alias GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags
Anti-GLA/alpha-Galactosidase A Antibody (2S483) is a Rabbit antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (2S483) can be used in WB.
Anti-GLA/alpha-Galactosidase A Antibody (2S483)
Anti-GLA/alpha-Galactosidase A Antibody (2S483)
😃Good
Catalog No. TMAY-02227Alias GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags
Anti-GLA/alpha-Galactosidase A Antibody (2S483) is a Rabbit antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (2S483) can be used in WB.
| Pack Size | Price | Availability | Quantity |
|---|---|---|---|
| 20 μL | $92 | 7-10 days | |
| 100 μL | $269 | 7-10 days |
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Chemical Properties
| Description | Anti-GLA/alpha-Galactosidase A Antibody (2S483) is a Rabbit antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (2S483) can be used in WB. |
| Alias | GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags |
| Ig Type | Monoclonal Rabbit IgG |
| Clone | 2S483 |
| Reactivity | Human |
| Verified Activity | Anti-GLA rabbit monoclonal antibody at 1:500 dilution. -Lane A: MCF7 Whole Cell Lysate. -Lane B: 293T Whole Cell lysate. -Lysates/proteins at 30 μg per lane. -Secondary -Goat Anti-Rabbit IgG H&L (Dylight800) at 1/10000 dilution. -Developed using the Odyssey technique. -Performed under reducing conditions. -Predicted band size:49 kDa. -Observed band size:49 kDa |
| Application | WB |
| Recommended Dose | WB: 1:500-1:2000 |
| Antibody Type | Monoclonal |
| Host Species | Rabbit |
| Construction | This antibody was obtained from a rabbit immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; TMPY-01727; NP_000160.1; Met1-Leu429). |
| Purification | Protein A |
| Appearance | Liquid |
| Formulation | 0.2 μm filtered solution in PBS |
| Research 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. |
| Conjucates | Unconjugated |
| Immunogen | Recombinant Human alpha-Galactosidase A / GLA protein (TMPY-01727) |
| Antigen Species | Human |
Stability & Storage
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free. |
| Transport | Shipping with blue ice. |
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Please see Inhibitor Handling Instructions for more frequently ask questions. Topics include: how to prepare stock solutions, how to store products, and cautions on cell-based assays & animal experiments, etc
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