ASL 抗体 (AA 301-400) (AbBy Fluor® 680)
Quick Overview for ASL 抗体 (AA 301-400) (AbBy Fluor® 680) (ABIN4996917)
抗原
See all ASL 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 301-400
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预测反应
- Human,Mouse,Rat,Dog,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 ASL/Argininosuccinate Lyase
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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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- ASL (Argininosuccinate Lyase (ASL))
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别名
- ASL/Argininosuccinate Lyase
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背景
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Synonyms: Argininosuccinase, Argininosuccinate lyase, Arginosuccinase, ARLY_HUMAN, ASAL, ASL, EC 4.3.2.1.
Background: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
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基因ID
- 435
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途径
- Response to Growth Hormone Stimulus
抗原
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