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Anti-Glucocerebrosidase 兔抗
Anti-Glucocerebrosidase antibody produced in rabbit
产品编号: | 3365055 |
规格: | ~1 mg/mL, affinity isolated antibody, buffered aqueous solution |
包装规格: | 200 μL |
产品类别: | 进口试剂 |
品牌: | Sigma-Aldrich |
优惠价: | 立即咨询 |
产品别名
Anti-Glucocerebrosidase 兔抗
Anti-Glucocerebrosidase antibody produced in rabbit
Anti-D-glucosyl-N-acylsphingosine glucohydrolase
Anti-Lysosomal glucocerebrosidase
Anti-GBA
Anti-Glucosidase, beta (Gluc)
Anti-GBA1
Anti-Glucosylceramidase (GlcCerase)
基本信息
NACRES | NA.41 |
General description【一般描述】 | GBA1 (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 βsheets, domain II with two β sheets making an immunoglobulin like domain and domain III with eight stranded β/α triosephosphate isomerase (TIM) barrel. |
Application【应用】 | Anti-Glucocerebrosidase antibody produced in rabbit has been used in western blotting and immunocytochemistry. Anti-Glucocerebrosidase antibody produced in rabbit is suitable for immunoblotting at a working concentration of 1-2μg/mL using HEK-293T cell lysate expressing human glucocerebrosidase (GBA). |
Biochem/physiol Actions【生化/生理作用】 | GBA (glucosidase, beta, acid) gene encodes a protein that cleaves the β-glucosidic linkage of glycosylceramide (GlcCer), an intermediate in glycolipid metabolism. Mutations in the human GBA gene cause a reduction in the GBA activity and accumulation of GlcCer in lysosomes of cells of the reticuloendothelial system. This causes Gaucher disease (GD), an inherited lysosomal storage disorder, characterised by severe loss of neurons in the central nervous system, fetal onset, hydrops fetalis, in utero fetal death and neonatal distress. It leads to changes in neuronal functionality including increased levels of tubular endoplasmic reticulum (ER) elements, a large increase in Ca2+ release from the ER in response to glutamate, and an increased sensitivity to glutamate-induced neurotoxicity. Mutations in the human GBA gene may contribute to the development of common age-related dementia known as dementia with Lewy bodies or DLB and may cause early-onset of Parkinson disease. |
Physical form【外形】 | Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide. |
产品性质
biological source【生物来源】 | rabbit |
Quality Level【质量水平】 | 200 |
conjugate【偶联物】 | unconjugated |
antibody form【抗体形式】 | affinity isolated antibody |
antibody product type | primary antibodies |
clone【克隆】 | polyclonal |
form【形式】 | buffered aqueous solution |
mol wt【分子量】 | antigen ~60 kDa |
species reactivity | rat, human, mouse |
enhanced validation【增强验证】 | recombinant expression Learn more about Antibody Enhanced Validation |
concentration【浓度】 | ~1 mg/mL |
technique(s) | western blot: 1-2 μg/mL using HEK-293T cell lysate expressing human glucocerebrosidase (GBA) or SH-SY-5Y cell lysate |
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) mouse ... Gba(14466) |
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 |
WGK | WGK 1 |
Personal Protective Equipment【个人防护装备】 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |