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(shù)支持           掃碼咨詢技術(shù)服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關于我們  聯(lián)系我們
在线观看片免费人成视频播放,天天干夜夜叉,就色干综合
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-BTD/BF594 Conjugated antibody (bs-11813R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11813R-BF594
英文名稱 Rabbit Anti-BTD/BF594 Conjugated antibody
中文名稱 BF594標記的生物素酶抗體
別    名 Biotinase; Biotinidase; Btd; Sp8; BTD_HUMAN; EC 3.5.1.12.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經(jīng)生物學  信號轉(zhuǎn)導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
產(chǎn)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 57kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Biotinidase
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(chǎn)品介紹 background:
Biotin, also known as vitamin B7, is an essential water-soluble vitamin that is a cofactor in glucogenesis and in the metabolism of fatty acids and leucine. Biotinidase is a 523 amino acid enzyme that catalyzes the hydrolysis of biocytin to biotin and lysine. Secreted into extracellular space, biotinidase is expressed in liver, heart, placenta, brain, skeletal muscle, pancreas and kidney. Biotinidase contains one carbon-nitrogen hydrolase domain, which is involved in the reduction of organic nitrogen compounds and ammonia production. Defects in the gene encoding biotinidase are the cause of biotinidase deficiency, which is characterized by skin rash, ataxia, seizures, hearing loss, hypotonia and optic atrophy. These symptoms are due to the individual’s inability to reutilize biotin and can, therefore, typically be treated with the addition of free biotin.

Function:
Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.

Subcellular Location:
Secreted.

DISEASE:
Defects in BTD are the cause of biotinidase deficiency (BTD deficiency) [MIM:253260]; also called late-onset multiple carboxylase deficiency. BTD deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis.
BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur.

Similarity:
Belongs to the CN hydrolase family. BTD/VNN subfamily.
Contains 1 CN hydrolase domain.

Database links:

Entrez Gene: 686 Human

Omim: 609019 Human

SwissProt: P43251 Human

Unigene: 517830 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權(quán)所有 2004-2026 www.kastlife.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)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(wǎng)安備110107000727號
主站蜘蛛池模板: 国产中文字幕在线播放 | 欧美精欧美乱码一二三四区 | 亚洲V国产V天堂A无码二区 | 久久a免费视频 | 一区两区三区四区乱码国产精品 | 中文字幕69| 久久久精品欧美 | 成人春色视频www | 中文字幕乱码中文乱码51精品 | 成人A片产无码免费奶头 | 久草免费在线视频 | 性xxxx乱又伦 | 97超碰人人干 | 亚洲激情校园 | 18禁黄无遮挡网站免费高清 | 国产99视频精品免费观看6 | 伊人久久国产 | 二次元人物桶二次元人物免费 | 欧美国产精品久久久 | 国产精品av无码毛片久久 | 国产福利一区二区精品秒拍 | 成人一级片免费看 | 国外精品久久久蜜桃免费全文阅读 | 欧美三级真做在线观看 | 亚洲欧美国产国产综合一区 | 无码人妻精品一区二区三 | 久久国产热这里只有精品 | 欧洲免费无线码在线一区 | 亚州日本乱码一区二区三区 | www.国产一区二区三区 | 伊人短视频 | 成人免费无码大片A毛片 | 92国产精品偷窥熟女精品视频 | 97色se| 蜜臀亚洲AV无码精品国产午夜. | 欧美亚洲日韩不卡在线在线观看 | 青草青青在线视频 | 欧美受xxxx黑人xyx爽 | 欧美xxxx色视频在线观看 | av影视在线播放 | 国产1区视频 |