亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

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
久久97精品久久久久久久看片,成人AV综合在线网站
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-BTD/BF488 Conjugated antibody (bs-11813R-BF488)
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-BF488
英文名稱 Rabbit Anti-BTD/BF488 Conjugated antibody
中文名稱 BF488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(lǐng)域 細胞生物  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 m.a6308.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
国语的ZOOFILIA杂交VIDEOS新 | 一区二区三区囯产精品乱码不卡 | 久久国产免费观看精品1 | 7788精品视频免费观看 | 91久久精品日日躁夜夜躁欧美 | 蹂躏清冷仙子翘臀雪乳 | 黄色网站在线免费观看 | 国产一级特黄aaa大片 | 国产无码三级片网址? | 消息称久青青国产高清视频 | 午夜亚洲WWW湿好爽 成人性大片免费观看网站YY | 日韩新片中文字幕无码 | 天天做天天摸天天爽天天爱 | 国产系列视频二区 | 人妻精品久久无码专区人妻 | 国内精品久久久久久久999下 | 东北女人一级毛片 | **色毛片免费观看 | 狼人久蕉在线播放 | 欧美精品狠狠色丁香婷婷 | 久久精品无码av一区二区 | 亚洲日韩一区精品射精 | 欧美久久久噜久噜久久XXⅩ交 | 亚洲欧洲中文日韩AV无码 | 高跟丝袜人妖ts被虐调教 | 五月天丁香婷深爱综合 | 中出人妻中文字幕无码 | 久久久久99狠狠综合久久 | 亚洲av日韩综合一区 | 国产成人精品免高潮费视频频 | 精品人妻aV中文字幕乱码 | 91久久精品无码一级毛片 | 国产高潮精品呻吟久久∧v无码 | 一牛影视传媒有限公司官网 | 亚洲最大的熟女水蜜桃AV网站 | 国产日产欧美一区二区蜜桃 | 三级毛片在线播放 | 久久综合亚洲鲁鲁五月天 | 国产91av在线播放网站 | 国产成人精品美女在线 | 亚洲国产精品成人AV无码久久综合网 |