FITC标记的常染色体隐性遗传性耳聋型36蛋白抗体
产品名称： FITC标记的常染色体隐性遗传性耳聋型36蛋白抗体
英文名称： Anti-Espin/DFNB36/FITC
产品编号： HZ-14635R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Espin/DFNB36/FITC Conjugated antibody
FITC标记的常染色体隐性遗传性耳聋型36蛋白抗体
| 英文名称 | Anti-Espin/DFNB36/FITC |
| 中文名称 | FITC标记的常染色体隐性遗传性耳聋型36蛋白抗体 |
| 别 名 | Autosomal recessive deafness type 36 protein; deafness autosomal recessive 36; DFNB36; ESPN_HUMAN; DKFZp434A196; DKFZp434G2126; Ectoplasmic specialization protein; ESPN; LP2654. |
| 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 发育生物学 神经生物学 信号转导 细胞骨架 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, 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 | 92kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Espin/DFNB36 |
| 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 °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. |
| 产品介绍 | 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 |
该基因编码多功能肌动蛋白结合蛋白。它在调节肌动蛋白纤维丰富的微绒毛型专门化的组织、尺寸、动力学和信号传递能力方面发挥着重要作用，这些专门化调节各种机械感觉和化学感觉细胞中的感官转导。该基因的突变与常染色体隐性神经感觉性耳聋、常染色体显性感觉神经性耳聋及无前庭病变有关。[ RefSeq，2009月11日]