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
九九国产精品入口麻豆,午夜熟女插插XX免费视频,性史性dvd影片农村毛片
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-PDCE2/AP Conjugated antibody (bs-19695R-AP)
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-19695R-AP
英文名稱 Rabbit Anti-PDCE2/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的丙酮酸脫氫酶E2抗體
別    名 Pyruvate Dehydrogenase E2; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; anti DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide; Dihydrolipoamide S Acetyltransferase; Dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex); Dihydrolipoamide S-Acetyltransferase; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial; DLAT; DLTA; E2; E2 component of pyruvate dehydrogenase complex; EC 2.3.1.12; M2 antigen complex 70 kDa subunit; M2 Antigen Complex 70kD Subunit; mitochondrial; ODP2_HUMAN; PBC; PDC E2; PDC-E2; PDCE2; Pyruvate dehydrogenase complex component E2; Pyruvate dehydrogenase complex E2 subunit; S acetyltransferase component of pyruvate dehydrogenase complex.  
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  神經生物學  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, Guinea Pig, Cat, ,Danio rerio)
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 63kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PDCE2
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:
This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009]

Function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Note=Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex.
Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.

Similarity:
Belongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.

Database links:

Entrez Gene: 512723 Cow

Entrez Gene: 1737 Human

Entrez Gene: 235339 Mouse

Entrez Gene: 397054 Pig

Entrez Gene: 81654 Rat

Entrez Gene: 324201 Zebrafish

Omim: 608770 Human

SwissProt: P11180 Cow

SwissProt: P10515 Human

SwissProt: Q8BMF4 Mouse

SwissProt: P08461 Rat

Unigene: 335551 Human

Unigene: 285076 Mouse

Unigene: 471144 Mouse

Unigene: 15413 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: 亚洲国产成人自拍 | 色播影院私人影院免费 | 成人午夜精品久久久久久久 | 少妇高潮无遮挡毛片免费播放 | 欧洲尺码日本尺码专线不卡顿 | 国产精品入口 | 国产在线拍偷自揄拍精品 | 日本成人片网站 | 在线视频亚洲欧美 | a级片免费在线播放 | 亚洲精品久久久久一区二区三区 | 四虎永久在线 | 女兵的真人大毛片 | 青青热久免费精品视频在线播放 | 撕开奶罩疯狂揉吮奶头 | 国产色欲色欲色欲WWW | 51cao在线观看| 亚洲一区二区二区久久成人婷婷 | 91夜夜夜| 精品久久99 | GOGOGO免费视频观看中文 | 麻豆视频成人 | 国产精品偷窥熟女精品视频 | 在线欧美色 | 波多野结衣系列18部无码观看a | 亚洲AV无码专区日韩乱码 | 国模吧久久 | cao榴 | 最新69国产成人精品视频免费 | 97精品超碰一区二区三区 | 色综合久久中文娱乐网 | 最新精品香蕉在线 | 中国成人网 | 午夜视频中文字幕 | 波多野结衣中文字幕在线视频 | 国产福利一区二区三区在线播放 | 日韩AV色综合网站 | 天堂久久久久VA久久久久 | 日韩成av人片在线观看 | 私人vps毛片 | 亚洲免费看黄 |