|IP||1-4 μL/mg of lysate|
**********Please Note: Optimal concentrations/dilutions should be determined by the end user.**********
Anti-GLA rabbit polyclonal antibody at 1:500 dilution
Lane A: 293T Whole Cell LysateLysates/proteins at 30 μg per lane.
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
GLA was immunoprecipitated using:
Lane A:0.5 mg 293T Whole Cell Lysate2 µL anti-GLA rabbit polyclonal antibody and 15 μl of 50 % Protein G agarose.Primary antibody:
Anti-GLA rabbit polyclonal antibody,at 1:200 dilutionSecondary antibody:
Clean-Blotô IP Detection Reagent (HRP) at 1:1000 dilutionDeveloped using the DAB staining technique.
Performed under reducing conditions.Predicted band size: 49 kDa
Observed band size: 52 kDa
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.
|Product Description||Host||Clonality||Application||Catalog# (PDF)|