產(chǎn)品編號 | bs-5015R |
英文名稱 | Rabbit Anti-ACADL antibody |
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 |
交叉反應(yīng) | Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Dog,Horse) |
產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,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 | 44kDa |
細胞定位 | 細胞漿 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 | 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 | |