Der Kaninchen Polyklonal anti-GLA Antikörper (Klon RB19115) (ABIN390687) detektiert spezifisch GLA in WB, FACS, IF und IHC (p).
Dieser Antikörper reagiert spezifisch mit Proben aus Human.
This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
Immunogen
This GLA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 83-112 amino acids from the N-terminal region of human GLA.
Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Lagerung
4 °C,-20 °C
Informationen zur Lagerung
Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
Haltbarkeit
6 months
Target
GLA
(Galactosidase, alpha (GLA))
Andere Bezeichnung
GLA
Hintergrund
GLA 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.