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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
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
3365055200 μL4730
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)
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