FITC标记的2号染色体开放阅读框39抗体上海沪震实业有限公司

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产品名称: FITC标记的2号染色体开放阅读框39抗体
英文名称: Anti-C2orf39/FITC
抗体货号: HZ-15149R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/9/14 9:24:00
详细资料:  实验方法技术资料
浏览人数:11
诚信指数:1084点
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
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产品详细描述

 Rabbit Anti-C2orf39/FITC Conjugated antibody

FITC标记的2号染色体开放阅读框39抗体

 

英文名称 Anti-C2orf39/FITC
中文名称 FITC标记的2号染色体开放阅读框39抗体
别    名 CC164_HUMAN; CCDC164; Coiled-coil domain-containing protein 164.   
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Sheep, 
产品应用 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 87kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C2orf39
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 癈 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癈. 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 癈.
产品介绍 background:
The second largest human chromosome, 2 consists of 237 million bases encoding over 1,400 genes and making up approximately 8% of the human genome. A number of genetic diseases are linked to genes on chromosome 2. Harlequin icthyosis, a rare and morbid skin deformity, is associated with mutations in the ABCA12 gene. The lipid metabolic disorder sitosterolemia is associated with ABCG5 and ABCG8. An extremely rare recessive genetic disorder, Alstr鰉 syndrome is due to mutations in the ALMS1 gene. Interestingly, chromosome 2 contains what appears to be a vestigial second centromere and vestigial telomeres which gives credence to the hypothesis that human chromosome 2 is the result of an ancient fusion of two ancestral chromosomes seen in modern form today in apes. The C2orf39 gene product has been provisionally designated C2orf39 pending further characterization.

Function:
Key component of the nexin-dynein regulatory complex(N-DRC), essential for N-DRC integrity. Required for the assemblyand regulation of specific classes of inner dynein arm motors. Mayalso function to restrict dynein-driven microtubule sliding, thusaiding in the generation of ciliary bending. 

Subcellular Location:
Cytoplasm, cytoskeleton, cilium axoneme (Bysimilarity).

DISEASE:
Note=Defects in CCDC164 are the cause of primary ciliarydyskinesia. A disorder characterized by abnormalities of motilecilia. Respiratory infections leading to chronic inflammation andbronchiectasis are recurrent, due to defects in the respiratorycilia; reduced fertility is often observed in male patients due toabnormalities of sperm tails. 

Similarity:
Belongs to the CCDC164 family. 

Database links:
UniProtKB/Swiss-Prot: Q96MC2.2

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

人类第二大染色体2由2.37亿个编码1400多个基因的碱基组成,约占人类基因组的8%。许多遗传疾病与2号染色体上的基因有关。丑角鳞癣,一种罕见的病态皮肤畸形,与ABCA12基因突变有关。脂类代谢紊乱是与ABCG5和ABCG8相关的。ALSTR综合征是一种极为罕见的隐性遗传性疾病,其原因在于ALMS1基因的突变。有趣的是,2号染色体含有看起来是残留的第二着丝粒和残留的端粒,这证实了人类2号染色体是今天在猿类身上看到的两个祖先染色体古代融合的结果。c2Orf39基因产物已暂时指定为C2OFF39,有待进一步鉴定。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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