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Rabbit Anti-CCDC50/BF647 Conjugated antibody (bs-6920R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-6920R-BF647
英文名稱 Rabbit Anti-CCDC50/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的卷曲螺旋結(jié)構(gòu)域蛋白50抗體
別    名 C3orf6; C3orf6 YMER; CCD50_HUMAN; CCDC 50; Ccdc50; Chromosome 3 open reading frame 6; Coiled coil domain containing 50; Coiled coil domain containing protein 50; Coiled-coil domain-containing protein 50; Protein Ymer; Ymer; Ymer protein.   
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 34kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CCDC50
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:
This gene encodes a soluble, cytoplasmic, tyrosine-phosphorylated protein with multiple ubiquitin-interacting domains. Mutations in this gene cause nonsyndromic, postlingual, progressive sensorineural DFNA44 hearing loss. In mouse, the protein is expressed in the inner ear during development and postnatal maturation and associates with microtubule-based structures. This protein may also function as a negative regulator of NF-kB signaling and as an effector of epidermal growth factor (EGF)-mediated cell signaling. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Oct 2008].

Function:
Involved in EGFR signaling.

Tissue Specificity:
Isoform 1 and isoform 2 are co-expressed in placenta, liver, lung, kidney and pancreas. Only isoform 1 is detected in skeletal muscle, brain and heart.

Post-translational modifications:
Phosphorylated on tyrosine residues.

DISEASE:
Defects in CCDC50 are the cause of deafness autosomal dominant type 44 (DFNA44) . A form of non-syndromic hearing loss. It is initially moderate and affects mainly low to mid frequencies. Later, it progresses to involve all the frequencies and leads to a profound hearing loss by the 6th decade. The onset of the hearing loss occurs in the first decade of life.

Database links:

Entrez Gene: 152137 Human

Entrez Gene: 67501 Mouse

Entrez Gene: 288022 Rat

SwissProt: Q8IVM0 Human

SwissProt: Q3TNK7 Mouse

SwissProt: Q3TRW1 Mouse

SwissProt: Q810U5 Mouse

SwissProt: Q810U0 Rat

Unigene: 478682 Human

Unigene: 258985 Mouse

Unigene: 9610 Rat



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