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Rabbit Anti-STRC/BF647 Conjugated antibody (bs-4466R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-4466R-BF647
英文名稱 Rabbit Anti-STRC/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的硬纖毛蛋白STRC抗體
別    名 DFNB16; Stereocilin; STRC; STRC_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat,  (predicted: Human, Mouse, Dog, Cow, Horse, Rabbit, )
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 193kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human STRC
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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 protein that is associated with the hair bundle of the sensory hair cells in the inner ear. The hair bundle is composed of stiff microvilli called stereocilia and is involved with mechanoreception of sound waves. This gene is part of a tandem duplication on chromosome 15; the second copy is a pseudogene. Mutations in this gene cause autosomal recessive non-syndromic deafness. [provided by RefSeq, Jul 2008]

Function:
Essential to the formation of horizontal top connectors between outer hair cell stereocilia.

Subcellular Location:
Cell surface. Associated with the hair bundle.

DISEASE:
Defects in STRC are the cause of deafness autosomal recessive type 16 (DFNB16) [MIM:603720]. DFNB16 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Defects in STRC are a cause of deafness-infertility syndrome (DIS) [MIM:611102]. DIS is characterized by deafness and infertility and is caused by large contiguous gene deletions at 15q15.3 that removes both STRC and CATSPER2 genes.

Similarity:
Belongs to the stereocilin family.

Database links:

Entrez Gene: 161497 Human

Entrez Gene: 140476 Mouse

Entrez Gene: 691937 Rat

Omim: 606440 Human

SwissProt: Q7RTU9 Human

SwissProt: Q8VIM6 Mouse

Unigene: 657395 Human



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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