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货号： bs-4466R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4466R-AP
- 英文名称
- Anti-STRC/AP
- 中文名称
- 碱性磷酸酶（AP）标记的硬纤毛蛋白STRC抗体
- 别 名
- DFNB16; Stereocilin; STRC; STRC_HUMAN.
- 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
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Cow, Horse, Rabbit,
- 产品应用
- WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 193kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human STRC
- 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 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: 161497Human
Entrez Gene: 140476Mouse
Entrez Gene: 691937Rat
Omim: 606440Human
SwissProt: Q7RTU9Human
SwissProt: Q8VIM6Mouse
Unigene: 657395Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.