產(chǎn)品編號(hào) | bs-5015R |
英文名稱 | ACADL Rabbit pAb |
中文名稱 | 酰基輔酶A脫氫酶長(zhǎng)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 |
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(cè)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 | |
| 国产精品视频免费看 | 亚洲6080yy久久无码产自国产· | 丝袜 美腿 性爱 电影 麻豆 | 香蕉大视频一二三区乱码 | 在线观看波多野结衣一区 | 丰满人妻熟女中文字幕 | 四川少如BBBBBBBBB | 在线观看永久免费麻豆 | 鲁大师在线观看日本影片 | 久久Y不卡人妻内射中出 | aV国产乱码一区二区三 | 寡妇高潮一级毛片免费看小说 | 日本老熟妇人妻妇毛多多 | 久久黄色视频可看中文无码 | 91精品人妻一区二区50路 | 国产一区二区三区在线h | 四虎精品成人免费网站 | 日本三级电影中文字幕 | 波多野结喷水最猛一部352 | 久久熟女人妻免费A片 | 国产小视频在线免费观看 | 浪潮av蜜臀av色欲av影.. | BBB片一毛片A片AA少妇 | 88欧美狠狠噜日日噜噜 | 黄色视频网站在线免费观看 | 中文乱码人妻一区二区三区视频 | 少妇高潮呻吟A片免费 | 白丝女疯狂 喷水自慰爽 | 无套内射在线观看 | 丰满老熟女一级AA片色欲 | 欧美日逼视频网站 | 亚洲AV片免费在线观看 | 四川少妇BBw搡BBBB槡BBBB 四川少妇bbw搡bbbb搡bbbb | 国产亚洲精品久久久久动 | 一级特黄a大片免费播放桃 国产乱国产乱老熟400部 | 3d动漫啪啪精品一区二区免费 | 欧美mv日韩mv国产网站 | 一级婬片试看15分钟 | 搡六十70老女人老熟女视频 | 特大巨人黑人AAA片BBC |