| 產(chǎn)品編號 | bs-5015R |
| 英文名稱 | ACADL Rabbit pAb |
| 中文名稱 | ?；o酶A脫氫酶長鏈抗體 |
| 別 名 | mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase. |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 轉(zhuǎn)錄調(diào)節(jié)因子 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 44 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ACADL: 201-300/430 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq]. Subunit: Homotetramer. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Similarity: Belongs to the acyl-CoA dehydrogenase family. SWISS: P28330 Gene ID: 33 Database links: Entrez Gene: 11363 Mouse Omim: 609576 Human SwissProt: P28330 Human SwissProt: P51174 Mouse Unigene: 471277 Human Unigene: 2445 Mouse Unigene: 174 Rat |
| 產(chǎn)品圖片 |
Sample:
Lane 1: Human Jurkat cell lysates
Lane 2: Human HeLa cell lysates
Lane 3: Human HepG2 cell lysates
Primary: Anti-ACADL (bs-5015R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 44 kDa
Observed band size: 48 kDa
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