GLA
Reaktivität: Human
IHC, ELISA
Wirt: Kaninchen
Polyclonal
unconjugated
Applikationshinweise
Optimal dilution of the Gla antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL
Beschränkungen
Nur für Forschungszwecke einsetzbar
Buffer
0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
Lagerung
-20 °C
Informationen zur Lagerung
After reconstitution, the Gla antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
Target
GLA
(Galactosidase, alpha (GLA))
Andere Bezeichnung
Gla / Galactosidase alpha
Hintergrund
Alpha-galactosidase is a glycoside hydrolase enzyme that encoded by the GLA gene. This gene is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.