97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
999久久a精品合区久久久,伊人久久蜜桃,久久人体rtys
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-ACADL/RBITC Conjugated antibody (bs-10150R-RBITC)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-10150R-RBITC
英文名稱 Rabbit Anti-ACADL/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的酰基輔酶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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADL
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
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.

Database links:

Entrez Gene: 33 Human

Entrez Gene: 614508 Cow

Entrez Gene: 11363 Mouse

Entrez Gene: 396931 Pig

Entrez Gene: 25287 Rat

Omim: 609576 Human

SwissProt: P28330 Human

SwissProt: P51174 Mouse

SwissProt: P79274 Pig

SwissProt: P15650 Rat

Unigene: 471277 Human

Unigene: 2445 Mouse

Unigene: 174 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 国产第一页福利影院 | 免费看毛片网站 | 国产精品成人av | 午煮香蕉小辣椒 | 亚洲欧洲无码一区二区三区 | 亚洲欧美日韩精品永久在线 | 亚洲国产精品久久久久久69 | 亚洲欧美不卡 | 精品国产一区二区三区麻豆小说 | 国产日韩欧美亚洲 | 日本正能量不良网站 | 国产一区二区精品久久岳 | 第一区在线观看免费国语入口 | 国产精品嫩草影院99网站 | 人人超碰人人爱超碰国产 | 国产亚洲精品欧洲在线观看 | 免费黄色av片 | 中文有码无码人妻在线 | 免费在线观看色视频 | 国产香蕉97碰碰视频VA碰碰看 | 日韩免费黄色片 | 午夜香吻高清观看视频在线 | 99久久人人爽亚洲精品美女 | 免费国产一区二区 | 一夲道av无码无卡免费 | 日本xxxx裸体xxxx视频大全 | 少妇无码一区二区三区 | 亚洲成人aa | 91porny九色在线 | 91亚洲国产在人线播放午夜 | 亚洲欧洲自拍拍偷无码 | 久久国产精品人妻丝袜 | www.亚洲成人| 婷婷成人丁香五月综合激情 | 久久久日韩精品一区二区 | 中文字幕永久视频 | 91看片| 日韩在线一二三 | 成人免费视频国一国二在线观看 | 亚洲精品久久无码AV片亚洲有色 | 免费网站看v片在线a |