Recombinant GBA 抗体
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北京 101111
Quick Overview for Recombinant GBA 抗体 (ABIN7828226)
抗原
See all GBA 抗体抗体类型
适用
宿主
克隆类型
标记
应用范围
克隆位点
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原理
- Recombinant GBA Monoclonal Antibody
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纯化方法
- Protein A purified
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免疫原
- Recombinant human GBA fragment
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亚型
- IgG, kappa
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anti-Glucosidase, Beta, Acid (GBA) (AA 146-235) antibody
GBA 适用: 人 WB, ELISA, IF, IHC (p) 宿主: 小鼠 Monoclonal 2E2 unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 117-214) antibodyGBA 适用: 人 WB, IHC, ICC, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) antibodyGBA 适用: 小鼠, 大鼠 IHC, IF 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 334-498) antibodyGBA 适用: 人 WB, IHC, ICC, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) antibodyGBA 适用: 人, 小鼠 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (C-Term) antibodyGBA 适用: 人 WB, ICC, IF, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 255-509) antibodyGBA 适用: 小鼠 WB, IHC, ICC, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 215-343) antibodyGBA 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 1-536) antibodyGBA 适用: 人 WB, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Beta, Acid (GBA) (AA 146-235) antibodyGBA 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 2H4 unconjugated
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应用备注
- WB 1:1000-1:20000,IHC 1:50
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限制
- 仅限研究用
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浓度
- 1 mg/mL
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缓冲液
- PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 % protein protectant.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.
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有效期
- 12 months
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- GBA (Glucosidase, Beta, Acid (GBA))
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别名
- GBA
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背景
- GBA,GBA1,GCB,GLUC,GC,9-O-sialyl GD3,7-O-sialyl GD3,ACID,acid (includes glucosylceramidase),beta,Acid beta glucosidase,Acid beta-glucosidase,Alglucerase,Beta glucocerebrosidase,BETA GLUCOSIDASE,betaGC,Beta-glucocerebrosidase,D glucosyl N acylsphingosine glucohydrolase,D-glucosyl-N-acylsphingosine glucohydrolase,EC 3.2.1.45,Gba protein,GCase,GLCM,Glucocerebrosidase,Glucocerebrosidase (alt.),GLUCOCEREBROSIDASE PSEUDOGENE,Glucosidase,Glucosidase beta,Glucosylceramidase,Imiglucerase,Lysosomal glucocerebrosidase,OTTHUMP00000033992,OTTHUMP00000033993,Beta-Glucosylceramidase (β-GC) is a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside into free ceramide and glucose. Lysosomal breakdown of glucocerebroside is required for cellular metabolism of complex lipids and proper turnover of cellular membrane. In the absence ofGBA, the gene that encodes β-GC, autophagic lysosome reformation is altered, suggesting that β-GC activity is critical to maintain functional lysosomes. The cellular function of lysosomes is to degrade and recycle cellular waste to maintain proper cellular energy metabolism. Mutations in humanGBAcause deficiency in β-GC, resulting in the accumulation of lysosomal glucocerebroside. Macrophages are particularly sensitive to lysosomal glucocerebroside accumulation due to their role in phagocytosis-mediated breakdown of cellular debris and dying cells. Gaucher disease, a rare autosomal recessive lysosomal storage disorder that is genetically linked toGBA, is marked by engorged ""Gaucher cell"" macrophages in the spleen, liver, and bone marrow. GBAmutations are the most common genetic risk factor for Parkinson's disease (PD), a neurodegenerative disease characterized by the loss of dopaminergic neurons in the substantia nigra with formation of α-synuclein-rich Lewy bodies in surviving neurons.GBAmutations may play a direct role in accumulation of α-synuclein by mechanisms that are poorly understood, but may include mislocalization of lysosomal β-GC causing impaired lysosomal degradation of α-synuclein. Cat.No. Product Name Clone No. IF:{{item.impact}} Journal:{{item.journal}} ({{item.year}}) DOI:{{item.doi}} Reactivity:{{item.species}} Sample Type:{{item.sample_type}} Previous {{ page }} Next Q{{(FAQpage.currentPage - 1)*pageSize+index+1}}:{{item.name}} Previous {{ page }} Next [
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分子量
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Calculated MW: 60 kDa
Observed MW: 60 kDa
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UniProt
- P04062
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途径
- Cellular Glucan Metabolic Process
抗原
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