强伦轩一级A片在线观看,中文字幕乱码人妻二区三区,鲁鲁狠狠狠7777一区二区,西西4444www无码精品
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(lián)系我們
搡BBB,搡BBBB,搡BBBB,韩国一级婬片A片AAA小说软件 ,日本一级婬片A片AAA毛多多
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-BTD/AP Conjugated antibody (bs-11813R-AP)
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-AP
英文名稱 Rabbit Anti-BTD/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)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 IHC-P=1:50-200 IHC-F=1:50-200 ICC=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.nmgps.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(yī)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(wǎng)安備110107000727號
再深点灬舒服灬受不了的播放地址 | 大黑人狂躁美女大BBBB小说 | 亚洲农村老熟妇肥BBBB | 蜜桃一区二区在线视频 | 在线国产一级视频 | 免费一级婬片A片AAA小说软件 | 国产精品资源在线 | 爱爱动态视频无码区免费看 | 成人一区二区三中文破解版新视 | 精品国产免费久久久久久桃子图片 | 人妻丰满精品一区二区三 | av高清无码在线观看 | aaa久久视频在线播放 | 成人AV一二区在线播放 | 91精品国产蜜臀色欲 | 国产在线观看精品 | 影音先锋AV成人资源站在线播放 | 天天AAA无码精品级 丝袜被扒在线观看网站 | 无码人妻熟好av又粗又大 | 91精品国产综合久久久久久漫画 | 欧美乱码熟妇色精精品 | 在线看黄色视频的网站 | 真人BBBBBBBBB毛片 | 91在线播放免费 | 在线免费观看国产三级片 | 红桃视频一区二区三区四区 | 国产日本美国在线视频观看视频 | 国产精品扒开脚做爽爽爽 | 国产成人秘 在线观看免费网站 | 韩国黑寡妇一级毛片 | 亚洲无码不卡毛片在线看 | 边啃奶边躁视频在线观看 | 特级婬片A片AAA毛片A级面粉 | 乱婬寡妇一区二区三区 | 永久免费看MV日本精品 | 国产又黄又大又粗视频 | 奇米影视一区二区三区 | 欧美性猛交XXXX黑人猛交 | 精品成人无码一区二区三区 | 亚洲AV无码乱码 | 国产精品a久久久久久 |