产品编号 | bs-7954R |
英文名称 | ALAD Rabbit pAb |
中文名称 | δ氨基乙酰丙酸脱水酶抗体 |
别 名 | ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; delta aminolevulinic acid dehydratase; delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase. |
研究领域 | 心血管 细胞生物 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
克 隆 号 | |
交叉反应 | Mouse, (predicted: Human, Rat, Dog, Cow, Horse, Rabbit, Sheep, ) |
产品应用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理论分子量 | 36 kDa |
检测分子量 | |
细胞定位 | 细胞核 细胞浆 细胞外基质 分泌型蛋白 |
性 状 | Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human ALAD: 151-240/330 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存条件 | Shipped at 4℃. Store at -20℃ 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. |
PubMed | PubMed |
产品介绍 |
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. Function: 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. Subunit: Homooctamer; active form. Homohexamer; low activity form. DISEASE: Defects in ALAD are the cause of acute hepatic porphyria (AHEPP) [MIM:612740]. 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. Similarity: Belongs to the ALADH family. SWISS: P13716 Gene ID: 210 Database links: Entrez Gene: 210 Human Entrez Gene: 17025 Mouse Omim: 125270 Human SwissProt: P13716 Human SwissProt: P10518 Mouse Unigene: 1227 Human Unigene: 6988 Mouse |
产品图片 | |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |