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
成人无码WWW免费视频网站,久久99热狠狠色精品一区,国产综合视频在线观看一区
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-BBS1/PE Conjugated antibody (bs-11507R-PE)
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-11507R-PE
英文名稱 Rabbit Anti-BBS1/PE Conjugated antibody
中文名稱 PE標記的巴爾得-別德爾綜合征相關(guān)蛋白5抗體
別    名 AI451249; Bardet-Biedl syndrome 1; Bardet-Biedl syndrome 1 homolog; Bardet-Biedl syndrome 1 protein; BBS1; BBS1_HUMAN; BBS2-like protein 2; D19Ertd609e.  
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)  內(nèi)分泌病  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, 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 65kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BBS5
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:
The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane.

Function:
This gene encodes a protein that has been directly linked to Bardet-Biedl syndrome. The primary features of this syndrome include retinal dystrophy, obesity, polydactyly, renal abnormalities and learning disabilities. Experimentation in non-human eukaryotes suggests that this gene is expressed in ciliated cells and that it is required for the formation of cilia. Alternate transcriptional splice variants have been observed but have not been fully characterized.

Subunit:
Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. Interacts with the C-terminus of RAB3IP. Interacts with CCDC28B.

Subcellular Location:
Cell projection

Tissue Specificity:
Highly expressed in the kidney. Also found in fetal tissue, testis, retina, adipose tissue, heart, skeletal muscle and pancreas.

DISEASE:
Defects in BBS1 are a cause of Bardet-Biedl syndrome type 1 (BBS1) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. Inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for disease manifestation in some cases (triallelic inheritance).

Database links:
UniProtKB/Swiss-Prot: Q8NFJ9.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

BBS蛋白是一類研究早期兒童肥胖綜合癥有關(guān)的其中一種。巴爾得-別德爾綜合征(Bardet-Biedl syndrome,BBS)的特征為不同程度的肥胖、智力延遲、色素視網(wǎng)膜病變、多指和腎臟異常。
版權(quán)所有 2004-2026 www.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
国产毛多水多高潮高清 | 黑人玩弄极品人妻系列 | 亚洲日韩国产一区二区蜜桃 | 91福利国产在线观一区二区 | 日本一区二区无卡高清视频 | 性一交一无一伦一精一品视频 | 免费精品国产自产拍在线观看图片 | 国产精品自产拍在线观看中文 | 欧美叉叉叉BBB网站 久久精品视频91 | 一级毛片视频免费观看 | 妖精视频一区二区免费 | 午夜夫妻试看120国产 | 欧美激情综合色综合啪啪五月 | A级毛片久久久久久精品 | 久久aa毛片免费播放嗯啊 | 精品无人乱码一区二区三区 | 内射国产老熟女综合导航 | 国产最新一区二区三区天堂 | 一本久久a久久精品免费不卡 | 日韩在线永久免费播放 | 亚洲国产成人AV网站 | 国产在线观看不卡 | 狼人香蕉香蕉在线28 - 百度 | 中文字幕大香视频蕉无码... | 精品久久久久成人码免费动漫 | 欧美一级毛片在播放免费 | 国产黄色视频在线观看 | 成年网站免费人性视频a站 老司机在线精品视频网站 亚洲色熟女图激情另类图区 | 欧美精品成人一区二区在线观看 | 日本一区二区三区在线网 | 亚洲第一区欧美国产不卡综合 | 亚洲无AV在线中文字幕 | 亚洲揄拍窥拍久久国产自揄拍 | 高清无码视频在线观看 | 青青草99久久精品国产综合 | XX性欧美肥妇精品久久久久久 | 国产又色又爽又刺激在线播放 | 精品一级毛片免费视频 | 日本大胆欧美人术艺术 | 日产亚洲一卡2卡3卡4卡网站 | 琪琪亚洲AV永久无码精品无码 |