97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

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
亚洲精品无码成案A片在线,国产成人自拍小视频,天堂资源
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-TCTN3/RBITC Conjugated antibody (bs-12321R-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-12321R-RBITC
英文名稱 Rabbit Anti-TCTN3/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的結構蛋白家族3抗體
別    名 C10orf61; Chromosome 10 open reading frame 61; DKFZP564D116; TCTN3; TECT3_HUMAN; Tectonic 3; Tectonic 3 precursor; Tectonic family member 3; Tectonic-3; TECT3; UNQ1881/PRO4324; PSEC0041.  
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  細胞凋亡  生長因子和激素  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, 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 64kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TCTN3/TECT3
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:
This gene encodes a member of the tectonic gene family which functions in Hedgehog signal transduction and development of the neural tube. Mutations in this gene have been associated with Orofaciodigital Syndrome IV and Joubert Syndrom 18. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Sep 2012].

Function:
Part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition (By similarity). May be involved in apoptosis regulation. Necessary for signal transduction through the sonic hedgehog (Shh) signaling pathway

Subunit:
Part of the tectonic-like complex (also named B9 complex) (By similarity).

Subcellular Location:
Membrane; Single-pass type I membrane protein (Potential).

DISEASE:
Defects in TCTN3 are the cause of orofaciodigital syndrome 4 (OFD4) [MIM:258860]. A form of orofaciodigital syndrome, a group of heterogeneous disorders characterized by malformations of the oral cavity, face and digits, and associated phenotypic abnormalities that lead to the delineation of various subtypes. OFD4 patients have tongue nodules, multiple frenulae, broad flat nose, hypertelorism, and short rib polydactyly with tibial dysplasia (Majewski syndrome). The presence of severe tibial aplasia differentiates OFD4 from OFD1. Additional features of cystic dysplastic kidneys and brain malformation, including occipital encephalocele, are observed in severely affected patients.
Defects in TCTN3 are the cause of Joubert syndrome 18 (JBTS18) [MIM:614815]. A form of Joubert syndrome, a disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. JBTS18 patients have vermis agenesis and the molar tooth sign as well as severe kyphoscoliosis. Other features include intrauterine growth retardation, oral anomalies, micrognathism, polydactyly and camptodactyly, joint laxity, horseshoe kidney, and ventricular septal defect. Note=TCTN3-mutated fibroblasts from JBTS18 patients fail to respond to Shh agonists suggesting that at least some of the defects in affected individuals may be secondary to reduced Shh signaling (PubMed:22883145).

Similarity:
Belongs to the tectonic family.

Database links:

Entrez Gene: 26123 Human

Entrez Gene: 67590 Mouse

Entrez Gene: 309486 Rat

SwissProt: Q6NUS6 Human

SwissProt: Q8R2Q6 Mouse

Unigene: 438991 Human

Unigene: 374056 Mouse

Unigene: 60758 Mouse



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 www.kastlife.cn 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: 日本国产黄色 | 亚洲日韩在线中文字幕线路2区 | 内射少妇一区27P | 无码高潮又爽又黄A片软件 18禁无遮挡免费视频网站 | 91日韩精品一区二区三区 | 久久九九热 | 一本色道无码道在线观 | 精品少妇一区二区三区在线播放 | 成年肉动漫在线观看无码 | 国产高清免费在线播放 | 成人综合婷婷国产精品久久蜜臀 | 久产久精九国品在线 | 欧美另类激情 | 九九热免费在线观看 | 欧美受xxxx黑人xyx爽 | 人人超碰人人爱超碰国产 | 亚洲国产精品综合小说图片区 | 国产精品视频区1 | 亚洲中文字幕国产综合 | 国产精品久久久尹人香蕉 | 欧美精品四区 | 一本之道高清无码视频 | 开心五月激情综合婷婷 | 中文字幕亚洲色妞精品天堂 | 精品国产一区二区三区久久久久 | 蜜桃久久精品一区二区 | 日本在线播放一二三区 | 国产一区二区三区四区成男人 | 精品无码一区二区的天堂 | 99热这里只有精品3 91热视频 | 香蕉久久久久久久AV网站 | 国产理论一区二区三区 | 亚洲中文字幕国产综合 | 国产视频不卡一区 | 午夜三级A三级三点窝 | 色呦呦久久久 | 中文字幕第9页 | 天天做天天爱夜夜爽女人爽 | 97国产情侣爱久久免费观看 | 无码人妻精品一区二区在线视频 | 久久综合狠狠综合久久综合88 |