OTC 抗体 (AA 51-150)
Quick Overview for OTC 抗体 (AA 51-150) (ABIN720505)
抗原
See all OTC 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 51-150
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交叉反应
- 人
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预测反应
- Mouse,Rat,Dog,Cow,Pig,Horse
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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 OTC
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亚型
- IgG
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应用备注
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WB 1:300-5000
ELISA 1:500-1000 -
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
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- OTC (Ornithine Carbamoyltransferase (OTC))
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别名
- OTC
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背景
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Synonyms: OCTD, Ornithine carbamoyltransferase, mitochondrial, Ornithine transcarbamylase, OTCase, OTC
Background: Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.
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基因ID
- 5009
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UniProt
- P00480
抗原
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