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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) |