FITC标记的常染色体隐性遗传性耳聋型31蛋白抗体上海沪震实业有限公司

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产品名称: FITC标记的常染色体隐性遗传性耳聋型31蛋白抗体
英文名称: Anti-DFNB31/FITC
抗体货号: HZ-
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: 2018/10/11 11:14:00
详细资料:  实验方法技术资料
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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-DFNB31/FITC Conjugated antibody

FITC标记的常染色体隐性遗传性耳聋型31蛋白抗体

 

英文名称 Anti-DFNB31/FITC
中文名称 FITC标记的常染色体隐性遗传性耳聋型31蛋白抗体
别    名 1110035G07Rik; Autosomal recessive deafness type 31 protein; CASK interacting protein CIP98; CIP 98; CIP98; Deafness autosomal recessive 31; DFNB 31; DKFZp434N014; KIAA1526; RP11 9M16.1; USH 2D; USH2D; Whirlin; WHRN; WHRN_HUMAN; WI.  
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  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Cow, Horse, Rabbit, 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 96kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DFNB31
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 is thought to function in the organization and stabilization of sterocilia elongation and actin cystoskeletal assembly, based on studies of the related mouse gene. Mutations in this gene have been associated with autosomal recessive non-syndromic deafness and Usher Syndrome. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms.[provided by RefSeq, Mar 2010]

Function:
Necessary for elongation and maintenance of inner and outer hair cell stereocilia in the organ of Corti in the inner ear.

Subcellular Location:
Cytoplasm. Cell projection > stereocilium. Cell projection > growth cone. Detected at the level of stereocilia in inner outer hair cells of the cochlea and vestibule. Co-localizes with the growing ends of actin filaments (By similarity). Colocalizes with MPP1 in the retina, at the outer limiting membrane (OLM), outer plexifirm layer (OPL), basal bodies and at the connecting cilium.

DISEASE:
Defects in WHRN are the cause of deafness autosomal recessive type 31 (DFNB31) [MIM:607084]. DFNB31 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 WHRN are the cause of Usher syndrome type 2D (USH2D) [MIM:611383]. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa and sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH2 is characterized by congenital mild hearing impairment with normal vestibular responses.

Similarity:
Contains 3 PDZ (DHR) domains.

Database links:

Entrez Gene: 25861 Human

Entrez Gene: 73750 Mouse

Entrez Gene: 313255 Rat

Omim: 607928 Human

SwissProt: Q9P202 Human

SwissProt: Q80VW5 Mouse

SwissProt: Q810W9 Rat

Unigene: 93836 Human

Unigene: 300397 Mouse

Unigene: 204268 Rat



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

根据对相关小鼠基因的研究,该基因被认为在星形纤毛伸长和肌动蛋白囊骨架组装的组织和稳定中起作用。该基因突变与常染色体隐性遗传非综合征性耳聋和阿瑟小子综合征有关。该基因的选择性剪接导致编码不同亚型的多个转录体变体。

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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