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WHRN Rabbit Polyclonal Antibody  Cite:0    分享

货号： IM58249
复制产品信息
  • 种属：H，M，R
  • 用途：WB
规格:
50μl 100μl
单位:
支
单价:￥1500.00
产品简介
储存与保存
注意事项
产品简介
  • 产品名称
    WHRN Rabbit Polyclonal Antibody
  • 别名
    DFNB31;KIAA1526
  • 类别
    抗体产品
  • 基因名称
    WHRN
  • 蛋白名称
    WHRN
  • Clonality
    Polyclonal
  • 推荐应用
    WB
  • 稀释度
    WB 1:500-2000
  • 反应种属
    Human,Mouse,Rat
  • 浓度
    1 mg/ml
  • 存储缓冲液
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
  • Human Gene ID
    25861
  • Human Swissprot No.
    Q9P202
  • Mouse Gene ID
    73750
  • Mouse Swissprot No.
    Q80VW5
  • Rat Gene ID
    313255
  • Rat Swissprot No.
    Q810W9
  • 免疫原
    Synthesized peptide derived from human WHRN AA range: 419-469
  • 特异性
    This antibody detects endogenous levels of WHRN at Human/Mouse/Rat
  • 参考分子量
    100kDa
  • 宿主
    Rabbit
  • 同种型
    Rabbit,IgG
  • 背景介绍
    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],
  • 细胞定位
    Cytoplasm . Cell projection, stereocilium . Cell projection, growth cone . Photoreceptor inner segment . Cell junction, synapse . Detected at the level of stereocilia in inner and outer hair cells of the cochlea and vestibule. Localizes to both tip and ankle-link stereocilia regions. Colocalizes with the growing ends of actin filaments. Colocalizes with MPP1 in the retina, at the outer limiting membrane (OLM), outer plexifirm layer (OPL), basal bodies and at the connecting cilium (CC). In photoreceptors, localizes at a plasma membrane microdomain in the apical inner segment that surrounds the connecting cilia called periciliary membrane complex. .
  • 功能
    disease:Defects in WHRN are the cause of non-syndromic sensorineural 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.,disease: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.,function:Necessary for elongation and maintenance of inner and outer hair cell stereocilia in the organ of Corti in the inner ear.,online information:Gene page,similarity:Contains 1 PDZ (DHR) domain.,similarity:Contains 3 PDZ (DHR) domains.,subcellular location: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.,subunit:Forms homooligomers. Binds CASK, MPP1/p55 and MYO15A via the C-terminal PDZ domain. Binding to MYO15A is necessary for localization of WHRN to stereocilia tips. Interacts with USH2A, GPR98/MASS1 and LRRC4C/NGL1.,
  • 纯化
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存与保存

1.保存：-20℃，Avoid freeze/thaw cycles

2.有效期：1年

注意事项

1. 本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。 

2. 为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。 

3. 实验结果可由多种因素影响，相关处理只限于产品本身，不涉及其他赔偿。 

 

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