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Monoclonal Anti-GBA antibody produced in mouse

品牌
Sigma-Aldrich
货号
WH0002629M1
规格纯度
clone 2e2, purified immunoglobulin, buffered aqueous solution
参考价格
5045.46 *本价格含增值税费
促销
服务
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数量
-+
产品名称:
Monoclonal Anti-GBA antibody produced in mouse
Anti-GLUC
Anti-glucosidase, beta; acid (includes glucosylceramidase)
Anti-GCB
Anti-GBA1
产品介绍:

产品说明

一般描述

This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants encoding the same protein. (provided by RefSeq)
GBA (β-glucocerebrosidase) gene is mapped to human chromosome 1q21. It is a lysosomal enzyme and is widely expressed. The protein has domain I with three‐stranded anti‐parallel β‐sheets, domain II with two β‐sheets making an immunoglobulin‐like domain and domain III with eight‐stranded β/αtriosephosphate isomerase (TIM) barrel.

免疫原

GBA (NP_000148, 146 a.a. ~ 235 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS

应用

Monoclonal Anti-GBA antibody has been used in immunofluorescence staining.

生化/生理作用

GBA (β-glucocerebrosidase) is responsible for the conversion of the glycolipid glucocerebroside to ceramide and glucose. Mutations in this gene are associated with Gaucher disease and Parkinson disease.

外形

Solution in phosphate buffered saline, pH 7.4

法律信息

GenBank is a registered trademark of United States Department of Health and Human Services

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

NACRESNA.41

产品性质

质量水平100
生物来源mouse
偶联物unconjugated
抗体形式purified immunoglobulin
antibody product typeprimary antibodies
克隆2e2, monoclonal
形式buffered aqueous solution
species reactivityhuman
technique(s)immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
indirect immunofluorescence: suitable
western blot: 1-5 μg/mL
同位素/亚型IgG2aκ
GenBank® accession no.NM_000157
UniProt登记号P04062
运输dry ice
储存温度−20℃
Gene Informationhuman ... GBA(2629)

安全信息

储存分类代码12 - Non Combustible Liquids
闪点(F)Not applicable
闪点(C)Not applicable
个人防护装备Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

Sigma-Aldrich

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