GBA (1P9) Rabbit Monoclonal Antibody-EnkiLife

GBA (1P9) Rabbit Monoclonal Antibody

规格：50μL 价格：￥1350
规格：100μL 价格：￥2250
应用（Application）：WB,IHC-P
种属（Reactivity）：Human
偶联物（Conjugate）：Unconjugated
基因名（Gene Name）：GBA SKU: AMRe11321 Category: 兔单克隆抗体 Tags: Human, WB, IHC-P, Unconjugated, Rabbit, -

说明书复制

产品概述

产品名称（Product Name）	GBA (1P9) Rabbit Monoclonal Antibody
描述（Description）	Recombinant rabbit monoclonal antibody
宿主（Host）	Rabbit
应用（Application）	WB,IHC
种属反应性（Reactivity）	Human

产品性能

偶联物（Conjugation）	Unconjugated
修饰（Modification）	Unmodified
同种型（Isotype）	IgG
克隆（Clonality）	Monoclonal
形式（Form）	Liquid
存放说明（Storage）	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
储存溶液（Buffer）	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% New type preservative N and 50% glycerol. Store at +4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle.
纯化方式（Purification）	Affinity purification

免疫原

基因名（Gene Name）	GBA
别名（Alternative Names）	Alglucerase; betaGC; GBA1; GCase; GCB; GLUC; Glucosylceramidase; Imiglucerase;
基因ID（Gene ID）	2629
蛋白ID（SwissProt ID）	P04062

产品应用

稀释比（Dilution Ratio）	WB 1:1000-1:5000,IHC 1:50-1:100
蛋白分子量（Molecular Weight）	60kDa

研究背景

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).