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- Bioss
- bs-7954R-Bio
- 2025年09月30日
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100ul
| 产品编号 | bs-7954R-Bio |
| 英文名称 | ALAD Rabbit pAb, Biotin conjugated |
| 中文名称 | Biotin标记的δ氨基乙酰丙酸脱水酶抗体 |
| 英文别名 | ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; delta aminolevulinic acid dehydratase; delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase. |
| 产品应用 | WB=1:500-2000 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human ALAD |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cardiovascular > Blood Cell Biology > Other Antibodies > Other Antibodies |
| 亚基 | Homooctamer; active form. Homohexamer; low activity form. |
| 相似性 | Belongs to the ALADH family. _x000D_ |
| 功能 | Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. |
| 保存条件 | Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.Involvement in disease:Defects in ALAD are the cause of acute hepatic porphyria (AHP). AHP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
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ALAD Rabbit pAb, Biotin conjugated(bs-7954R-Bio)-100ul
￥2980
