GBA (1P9) Rabbit Monoclonal Antibody, Unconjugated

Product Overview

• Article Name: GBA (1P9) Rabbit Monoclonal Antibody, Unconjugated
• Biozol Catalog Number: EKL-AMRE11321
• Supplier Catalog Number: AMRe11321
• Alternative Catalog Number: EKL-AMRE11321-50UL, EKL-AMRE11321-100UL, EKL-AMRE11321-200UL
• Manufacturer: EnkiLife
• Host: Rabbit
• Category: Antikörper
• Application: IHC, WB
• Species Reactivity: Human
• Conjugation: Unconjugated
• Alternative Names: Alglucerase, betaGC, GBA1, GCase, GCB, GLUC, Glucosylceramidase, Imiglucerase,

Product Description

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).

Product Properties

• Clonality: Monoclonal
• Molecular Weight: 60kDa
• NCBI: 2629
• UniProt: P04062
• Buffer: 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.
• Purity: Affinity purification
• Form: Liquid
• Target: GBA

Application Notes

• Application Dilute: WB 1:1000-1:5000,IHC 1:50-1:100