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
国产亚洲精品自在线观看 ,国产精品视频一区国模私拍
Rabbit Anti-BTD/Biotin Conjugated antibody (bs-11813R-Bio)
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-Bio
英文名稱 Rabbit Anti-BTD/Biotin Conjugated antibody
中文名稱 生物素標(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 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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
色欲AV综合AV无码AⅤ | 精人妻无码一区二区三区 | 最近中文字幕大全在线电影视频 | 国产AV一区二区三区日韩 | 久久综合亚洲鲁鲁五月天 | 亚洲a欧美v在线观看 | 国精产品999国精产品视频 | A级毛片无码久久精品免费 九九视频精品全部免费播放 | 亚洲中文字幕无码一区在线 | 99精品无码一区二区毛片免费 | 欧美日韩精品视频一区二区三区在线观看 | 亚洲精品国产综合久久一线 | **一级毛片全部免 | 色哟哟国产精品一区二区真心好看 | 中出人妻中文字幕无码 | 亚洲AV永久久久久久久无码精品 | 国产又粗又猛又爽又黄的视频 | 无码AV免费精品一区二区三区 | 精品少妇人妻AV一区二区三区 | 成人影片麻豆国产影片免费观看 | 又爽又高潮的BB视频免费看 | 2020欧美精品A∨ | 国产欧美一区二区三区免费 | 中文字幕亚洲综合小综合 | 少妇高潮太爽了在线视频 | 免费麻豆国产黄网站在线观看 | 亚洲精品无码专区在线播放 | 欧美大片AAAAA免费观看 | 国产黄色一级网站 | 久青草久青草视频在线观看 | AA免费观看的1000部电影 | 国产乱妇乱子在线视频 | 日韩国产精品一区二区三区 | 丰满少妇又硬又粗又大视频 | 无码人妻熟妇av又粗又大 | 亚洲中文字幕无码久久1 | 久久久久亚洲精品天堂久久久久久 | 精品少妇一区二区激情在线 | 风韵丰满熟妇啪啪区老熟熟女 | 日本一区午夜爱爱 | 91青青国产在线观看免费 |