GCS1 抗体 (N-Term)
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北京 101111
Quick Overview for GCS1 抗体 (N-Term) (ABIN389016)
抗原
See all GCS1 (MOGS) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- AA 1-30, N-Term
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纯化方法
- This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
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免疫原
- This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human GCS1.
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亚型
- Ig Fraction
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应用备注
- WB: 1:1000
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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有效期
- 6 months
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- GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))
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别名
- GCS1
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背景
- GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
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分子量
- 91918
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基因ID
- 7841
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NCBI登录号
- NP_001139630, NP_006293
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UniProt
- Q13724
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途径
- SARS-CoV-2 Protein Interactome
抗原
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