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Monoclonal Anti-GBA antibody produced in mouse

产品编号:3145481
规格:clone 2e2, purified immunoglobulin, buffered aqueous solution
包装规格:100 μG
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
3145481100 μG5040
产品别名

Monoclonal Anti-GBA antibody produced in mouse

Anti-GLUC

Anti-glucosidase, beta; acid (includes glucosylceramidase)

Anti-GCB

Anti-GBA1

基本信息
NACRES
NA.41
General description【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.
Immunogen【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
Application【应用】
Monoclonal Anti-GBA antibody has been used in immunofluorescence staining.
Biochem/physiol Actions【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.
Physical form【外形】
Solution in phosphate buffered saline, pH 7.4
Legal Information【法律信息】
GenBank is a registered trademark of United States Department of Health and Human Services
产品性质
Quality Level【质量水平】
100
biological source【生物来源】
mouse
conjugate【偶联物】
unconjugated
antibody form【抗体形式】
purified immunoglobulin
antibody product type
primary antibodies
clone【克隆】
2e2, monoclonal
form【形式】
buffered aqueous solution
species reactivity
human
technique(s)
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
indirect immunofluorescence: suitable
western blot: 1-5 μg/mL
isotype【同位素/亚型】
IgG2aκ
GenBank® accession no.
NM_000157
UniProt accession no.【UniProt登记号】
P04062
shipped in【运输】
dry ice
storage temp.【储存温度】
−20℃
Gene Information
human ... GBA(2629)
产品说明
Disclaimer【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.
安全信息
Storage Class Code【储存分类代码】
12 - Non Combustible Liquids
Personal Protective Equipment【个人防护装备】
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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