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OTC 抗体 (AA 51-150)

This anti-OTC antibody is a 兔 多克隆 antibody detecting OTC in WB 和 ELISA. Suitable for 人.
产品编号 ABIN720505
发货至: 中国

Quick Overview for OTC 抗体 (AA 51-150) (ABIN720505)

抗原

See all OTC 抗体
OTC (Ornithine Carbamoyltransferase (OTC))

适用

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宿主

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克隆类型

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  • 10
多克隆

标记

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This OTC antibody is un-conjugated

应用范围

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Western Blotting (WB), ELISA
  • 抗原表位

    • 15
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    AA 51-150

    交叉反应

    预测反应

    Mouse,Rat,Dog,Cow,Pig,Horse

    纯化方法

    Purified by Protein A.

    免疫原

    KLH conjugated synthetic peptide derived from human OTC

    亚型

    IgG
  • 应用备注

    WB 1:300-5000
    ELISA 1:500-1000

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

    储存液

    ProClin

    注意事项

    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

    储存条件

    4 °C,-20 °C

    储存方法

    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

    有效期

    12 months
  • 抗原

    OTC (Ornithine Carbamoyltransferase (OTC))

    别名

    OTC

    背景

    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.

    基因ID

    5009

    UniProt

    P00480
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