GMPPB 抗体 (AA 111-210) (AbBy Fluor® 750)
Quick Overview for GMPPB 抗体 (AA 111-210) (AbBy Fluor® 750) (ABIN5003702)
抗原
See all GMPPB 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 111-210
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预测反应
- Human,Mouse,Rat,Cow,Sheep,Pig,Horse,Rabbit
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human GMPPB
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亚型
- IgG
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应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- GMPPB (GDP-Mannose Pyrophosphorylase B (GMPPB))
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别名
- GMPPB
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背景
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Synonyms: GDP mannose pyrophosphorylase B, GDP-mannose pyrophosphorylase B, GMPPB, GMPPB_HUMAN, GTP-mannose-1-phosphate guanylyltransferase beta, KIAA1851, Mannose 1 phosphate guanylyltransferase, Mannose-1-phosphate guanyltransferase beta.
Background: GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events.
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基因ID
- 29925
抗原
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