超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

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
成人av一区二区亚洲精,日批免费观看,狠狠香蕉
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-ACADL/PE Conjugated antibody (bs-5015R-PE)
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-5015R-PE
英文名稱 Rabbit Anti-ACADL/PE Conjugated antibody
中文名稱 PE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, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 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.ggggww.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: 91骚片 | 中国人与拘一级毛片 | 黄在线观看网站 | 国产真实野战在线视频 | 成人在线中文字幕 | 继承者们在线观看免费高清韩剧 | 美淫在线 | 国产亚洲影视在线视频播放 | 国产va亚洲va在线va | 亚洲日韩制服丝袜中文字幕 | 97人伦色伦成人免费视频 | 99精品免费视频 | 男人女人真曰批的视频8 | 国产91av在线| 一区二区国产精品视频 | 免费阿v视频 | 护士hd白色free性xxxⅹ | 国产色黄| 亚洲精品无码不卡在线播放 | 国产精品国产三级国产普通话一 | 337P日本欧洲亚洲大胆 | 天堂中文在线最新版WWW | 亚洲s色大片| 日本丰满大乳免费xxxx | 日本四虎影院 | 欧美成人二区 | 日韩精品亚洲专区在线观看 | 种付おじさんと在线观看 | av免费观看网页 | 69xx视频在线观看 | 亚洲国产成人av在线 | 国产亚洲综合AA系列 | 午夜国产情侣在线播放 | 美女张开腿让男生桶视频 | 日韩久久久久久久久久久久 | 国产美女精品AⅤ在线播放 日本三区三区三区二区二区二区高清在线 | 国产成年人小视频 | 国产成人无码精品久免费 | 精品少妇一区二区三区在线视频 | 国产成人精品日本亚洲网站 | 芒果视频91 |