GBA (1P9) Rabbit Monoclonal Antibody, Unconjugated
Artikelnummer:
EKL-AMRE11321
Produktübersicht
Artikelname:
GBA (1P9) Rabbit Monoclonal Antibody, Unconjugated
Artikelnummer:
EKL-AMRE11321
Hersteller Artikelnummer:
AMRe11321
Alternativnummer:
EKL-AMRE11321-50UL, EKL-AMRE11321-100UL, EKL-AMRE11321-200UL
Hersteller:
EnkiLife
Wirt:
Rabbit
Kategorie:
Antikörper
Applikation:
IHC, WB
Spezies Reaktivität:
Human
Konjugation:
Unconjugated
Alternative Synonym:
Alglucerase, betaGC, GBA1, GCase, GCB, GLUC, Glucosylceramidase, Imiglucerase,
Produktbeschreibung
Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800], also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed: 24211208, PubMed: 15916907). Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed: 27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed: 19279011). Also plays a role in cholesterol metabolism (PubMed: 24211208, PubMed: 26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed: 24211208, PubMed: 26724485). The short chain saturated C8:0- GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed: 24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed: 26724485). Finally, may also hydrolyze cholesteryl- beta-D-glucoside to produce D-glucose and cholesterol (PubMed: 24211208, PubMed: 26724485).
Produkteigenschaften
Klonalität:
Monoclonal
Molekulargewicht:
60kDa
NCBI:
2629
UniProt:
P04062
Puffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% New type preservative N and 50% glycerol. Store at +4C short term. Store at -20C long term. Avoid freeze / thaw cycle.
Reinheit:
Affinity purification
Formulierung:
Liquid
Target-Kategorie:
GBA
Anwendungsbeschreibung
Application Verdünnung:
WB 1:1000-1:5000,IHC 1:50-1:100
