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Rabbit Anti-Espin/DFNB36/FITC Conjugated antibody (bs-14635R-FITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-14635R-FITC
英文名稱 Rabbit Anti-Espin/DFNB36/FITC Conjugated antibody
中文名稱 FITC標記的常染色體隱性遺傳性耳聾型36蛋白抗體
別    名 Autosomal recessive deafness type 36 protein; deafness autosomal recessive 36; DFNB36; ESPN_HUMAN; DKFZp434A196; DKFZp434G2126; Ectoplasmic specialization protein; ESPN; LP2654.  
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  發育生物學  神經生物學  信號轉導  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, 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 92kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Espin/DFNB36
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 multifunctional actin-bundling protein. It plays a major role in regulating the organization, dimensions, dynamics, and signaling capacities of the actin filament-rich, microvillus-type specializations that mediate sensory transduction in various mechanosensory and chemosensory cells. Mutations in this gene are associated with autosomal recessive neurosensory deafness, and autosomal dominant sensorineural deafness without vestibular involvement. [provided by RefSeq, Nov 2009]

Function:
Espin is a multifunctional actin bundling protein. It plays a major role in regulating the organization, dimensions, dynamics and signaling capacities of the actin filament rich, microvillus type specializations that mediate sensory transduction in various mechanosensory and chemosensory cells.

Subunit:
Monomer (By similarity). Binds F-actin in a Ca(2+)-resistant fashion (By similarity). Interacts (via N-terminal) with BAIAP2 (via SH3-domain) (By similarity). Interacts with PFN2 (By similarity).

Subcellular Location:
Cytoplasm, cytoskeleton. Cell projection, stereocilium. Cell projection, microvillus.

DISEASE:
Deafness, autosomal recessive, 36, with or without vestibular involvement (DFNB36) [MIM:609006]: A form of non-syndromic 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. DFNB36 is characterized by prelingual, profound hearing loss, and vestibular areflexia in some patients. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Contains 9 ANK repeats.
Contains 1 WH2 domain.

Database links:

Entrez Gene: 83715 Human

Omim: 606351 Human

SwissProt: B1AK53 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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