精品国产av自拍_精品日韩欧美在线视频一区二区_一区二区在线观看在线_福利在线观看免费高清完整版_无码免费动漫老黄网站_无码一区在线观看视频_精品五月精品婷婷_免费国产日本高清_亚洲特黄特色一级在线观看_国产Ⅴ亚洲Ⅴ欧美Ⅴ专区

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
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(guān)于我們  聯(lián)系我們
思思久久er99精品亚洲,特级毛片在线大全免费播放
Rabbit Anti-BTD/Cy5.5 Conjugated antibody (bs-11813R-Cy5.5)
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-Cy5.5
英文名稱 Rabbit Anti-BTD/Cy5.5 Conjugated antibody
中文名稱 Cy5.5標(biāo)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(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)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 0592123.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
最新国学生自产拍短视频 | 无码人妻熟妇av又粗又大 | 久久久99精品免费观看 | 国产人成无码视频在线1000 | 成全高清视频免费观看动漫版 | 亚洲欧美自拍另类图片色 | 国产综合无码一区二区色蜜蜜 | 亚洲中久无码永久在线观看软件 | 色婷婷欧美在线播放内射 | 男女无遮挡XX00动态图120秒 | 国产日韩欧美三级 | 欧美人与性囗牲恔配 | 亚洲欧美中文字幕5发布 | 久久精品极品盛宴观看 | 奶头好大揉着好爽gif动态 | 国产黄A真人一级无码毛片一区二区三区 | 亚洲中文字幕无码中文字无摸 | 又大又硬又爽18禁免费看 | 日韩欧美群交P内射捆绑 | 五月天婷五月天综合网 | 国产AAA片精品网站 打扑克污黄对网站在线看 男女激情射精国产精品久久 | 国产喷水1区2区3区咪咪爱AV | 亚洲AV成人一区二区三区在线看 | 超碰国产精品人人做人人爱 | 亚洲精品一二三区区别在哪 | 一级特黄AAA大片在线观看 | 成人免费一级毛片在线播放视频 | 欧美激情视频二区 | 色成人网站WWW永久在线观看 | 成人午夜福利视频后入 | 国产午夜无码精品免费看性色 | 麻豆精品传媒一二三区 | 成人网站国产在线视频内射视频 | 一级做a爰片久久毛片免费陪 | 亚洲天天在线日亚洲洲精 | 成人永久免费国产乱码二区三区视频在线 | 亚洲国产日本一区二区 | 国产精品久久国产三级国电话系列 | 一级毛片免费在线观看网站 | 国产男女性潮高清免费网站 | 国产主播AV福利精品一区 |