麻豆av资源_日本三级一区_www.狠狠艹_国产精妇在线观看第一区_成人性生交大片免费看中国A片_日本一本久久

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
亚洲一级性生活,亚洲精品免费av,欧美秋霞
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-BTD/RBITC Conjugated antibody (bs-11813R-RBITC)
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-11813R-RBITC
英文名稱 Rabbit Anti-BTD/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的生物素酶抗體
別    名 Biotinase; Biotinidase; Btd; Sp8; BTD_HUMAN; EC 3.5.1.12.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, )
產品應用 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.
版權所有 2004-2026 m.jywcc.com 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: 综合视频一区二区三区 | 国产欧美亚洲精品第一区软件 | 97超碰精品成人国产 | 日韩激情一区二区 | 欧美啊啊啊视频 | 国产精品一区12p | avhd老司机101| 中国特黄视频 | 国产毛片a高清日本在线 | 久久女同互慰一区二区三区 | 日韩av免费网址 | 一面亲着一面膜下奶韩剧放下 | 欧美乱偷一区二区三区在线 | 91精品国产影片一区二区三区 | av片免费 | 强开少妇嫩苞又嫩又紧九色 | av最新资源在线 | 中文字幕在线看 | 少妇愉情理伦片丰满丰满午夜 | 久久久久高潮综合影院 | 四虎私人影院 | 一本一道久久a久久精品 | 成人网在线看 | 亚洲草逼 | 高清精品自拍亚洲 | 久久a视频 | 婷婷影院在线综合免费视频 | 强行征服邻居人妻 | 精品人妻无码一区二区三区软件 | 巨尻av| 狠狠色亚洲 | 国产精品成人网 | 四虎综合网 | 夜夜夜夜夜夜久久久久久久 | 无码AV一级毛片在线播放 | 欧美怡红院免费全部视频 | 夜夜操夜夜骑 | 亚洲色成人一区二区三区小说 | 日韩中文字幕网站 | 亚洲欧美国产日韩一区夜色 | 天天狠天天透天天伊人 |