OTOA,耳聋、常染色体隐性遗传22抗体

产品编号HZ-11060R
英文名称OTOA
中文名称耳聋、常染色体隐性遗传22抗体
别 名Cancer/testis antigen 108; CT108; Deafness, autosomal recessive 22; DFNB22; OTOA; OTOAN_HUMAN; Otoancorin.
说 明 书0.1ml 0.2ml
研究领域神经生物学 细胞粘附分子 细胞外基质
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat,
OTOA,耳聋、常染色体隐性遗传22抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量122kDa
细胞定位细胞膜 细胞外基质 分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human OTOA/DFNB22
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.
OTOA,耳聋、常染色体隐性遗传22抗体PubMedPubMed
产品介绍background:
Otoancorin, also known as OTOA, CT108 or DFNB22, is a 1,153 amino acid protein belonging to the stereocilin family. Expressed in the inner ear and restricted to the interface between the apical surface of sensory epithelia, otoancorin is suggested to act as an adhesion molecule. Otoancorin ensures the attachment of the inner ear acellular gels to the apical surface of the underlying nonsensory cells. Mutations in the gene encoding otoancorin leads to deafness autosomal recessive type 22 (DFNB22), which 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. Existing as three alternatively spliced isoforms, otoancorin is encoded by a gene located on human chromosome 16p12.2.

Function:
May act as an adhesion molecule.

Subcellular Location:
Apical cell membrane. Secreted > extracellular space > extracellular matrix. At the interface between the apical surface of the epithelia and the overlying acellular gel of the tectorial and otoconial membranes.

DISEASE:
Defects in OTOA are the cause of deafness autosomal recessive type 22 (DFNB22) [MIM:607039]. DFNB22 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.

Similarity:
Belongs to the stereocilin family.

Database links:
Entrez Gene: 146183 Human
Entrez Gene: 246190 Mouse
Omim: 607038 Human
SwissProt: Q7RTW8 Human
SwissProt: Q8K561 Mouse
Unigene: 408336 Human

OTOA,耳聋、常染色体隐性遗传22抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.