感觉神经性耳聋常染色体隐性遗传61抗体

英文名称    Slc26a5    

中文名称    感觉神经性耳聋常染色体隐性遗传61抗体    

别    名    Prestin; Deafness neurosensory autosomal recessive 61; DFNB 61; DFNB61; MGC118886; MGC118887; MGC118888; MGC118889; PRES; Prestin (motor protein); Prestin; S26A5_HUMAN; SLC26A5; Solute carrier family 26 member 5 (prestin); Solute carrier family 26 member 5.    

感觉神经性耳聋常染色体隐性遗传61抗体       

说 明 书    0.2ml      

研究领域    细胞生物      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=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    81kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Slc26a5    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

感觉神经性耳聋常染色体隐性遗传61抗体 产品介绍    background:
This gene encodes a member of the SLC26A/SulP transporter family. The protein functions as a molecular motor in motile outer hair cells (OHCs) of the cochlea, inducing changes in cell length that act to amplify sound levels. The transmembrane protein is an incomplete anion transporter, and does not allow anions to cross the cell membrane but instead undergoes a conformational change in response to changes in intracellular Cl- levels that results in a change in cell length. The protein functions at microsecond rates, which is several orders of magnitude faster than conventional molecular motor proteins. Mutations in this gene are potential candidates for causing neurosensory deafness. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2009]

Function:
Motor protein that converts auditory stimuli to length changes in outer hair cells and mediates sound amplification in the mammalian hearing organ. Prestin is a bidirectional voltage-to-force converter, it can operate at microsecond rates. It uses cytoplasmic anions as extrinsic voltage sensors, probably chloride and bicarbonate. After binding to a site with millimolar affinity, these anions are translocated across the membrane in response to changes in the transmembrane voltage. They move towards the extracellular surface following hyperpolarization, and towards the cytoplasmic side in response to depolarization. As a consequence, this translocation triggers conformational changes in the protein that ultimately alter its surface area in the plane of the plasma membrane. The area decreases when the anion is near the cytoplasmic face of the membrane (short state), and increases when the ion has crossed the membrane to the outer surface (long state). So, it acts as an incomplete transporter. It swings anions across the membrane, but does not allow these anions to dissociate and escape to the extracellular space. Salicylate, an inhibitor of outer hair cell motility, acts as competitive antagonist at the prestin anion-binding site (By similarity). 

Subcellular Location:
Cell membrane; Multi-pass membrane protein. Note=Lateral wall of outer hair cells. 

DISEASE:
Deafness, autosomal recessive, 61 (DFNB61) [MIM:613865]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. 

Similarity:
Belongs to the SLC26A/SulP transporter (TC 2.A.53) family.
Contains 1 STAS domain. 

Gene ID:
375611

Database links:

Entrez Gene: 375611 Human

Omim: 604943 Human

SwissProt: P58743 Human

Unigene: 585146 Human

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

感觉神经性耳聋常染色体隐性遗传61抗体 

hz-0336R-hzy5 Rabbit Anti-MK IgM/Cy5  Cy5标记的兔抗猴IgM

hz-0336R-hzy5.5 Rabbit Anti-MK IgM/Cy5.5  Cy5.5标记的兔抗猴IgM

hz-0336R-hzy7 Rabbit Anti-MK IgM/Cy7  Cy7标记的兔抗猴IgM

hz-0336R-PE Rabbit Anti-MK IgM/PE  PE标记的兔抗猴IgM

hz-0336R-APhz Rabbit Anti-MK IgM/APC  APC标记的兔抗猴IgM

hz-0336R-AF350 Rabbit Anti-MK IgM/Alexa Fluor 350  Alexa Fluor 350标记的兔抗猴IgM

hz-0336R-AF488 Rabbit Anti-MK IgM/Alexa Fluor 488  Alexa Fluor 488标记的兔抗猴IgM

hz-0336R-AF555 Rabbit Anti-MK IgM/Alexa Fluor 555  Alexa Fluor 555标记的兔抗猴IgM

hz-0336R-AF647 Rabbit Anti-MK IgM/Alexa Fluor 647  Alexa Fluor 647标记的兔抗猴IgM

hz-0336R-PE-hzy5.5 Rabbit Anti-MK IgM/PE-Cy5.5  PE-Cy5.5标记的兔抗猴IgM

hz-0336R-PE-hzy7 Rabbit Anti-MK IgM/PE-Cy7  PE-Cy7标记的兔抗猴IgM

hz-0336R-Bio Rabbit Anti-Monkey IgM/Bio  生物素标记的兔抗猴IgM

hz-0336R-AP Rabbit Anti-Monkey IgM/AP  碱性磷酸酶（AP）标记的兔抗猴IgM

hz-0336R-Gold Rabbit Anti-Monkey IgM/Gold  胶体金标记的兔抗猴IgM

hz-0336R-HRP Rabbit Anti-Monkey IgM/HRP  辣根过氧化物酶标记的兔抗猴IgM

hz-0336R-PE-hzy3 Rabbit Anti-Monkey IgM/PE-Cy3  PE-Cy3标记的兔抗猴IgM

hz-0336R-PE-hzY5 Rabbit Anti-Monkey IgM/PE-CY5  PE-CY5标记的兔抗猴IgM

hz-0336R-FIThz Rabbit Anti-Monkey IgM/FITC  FITC标记的兔抗猴IgM

hz-0336R-RBIThz Rabbit Anti-Monkey IgM/RBITC  罗丹明标记的兔抗猴IgM

hz-0296R-RBIThz Rabbit Anti-mouse IgG/RBITC  罗丹明标记的兔抗小鼠IgG

hz-0296R-FIThz Rabbit Anti-mouse IgG/FITC  FITC标记的兔抗小鼠IgG

hz-0296R-Gold Rabbit Anti-mouse IgG/Gold  胶体金标记的兔抗小鼠IgG

hz-0296R-HRP Rabbit Anti-mouse IgG/HRP  辣根过氧化物酶标记的兔抗小鼠IgG

hz-0296R-AP Rabbit Anti-mouse IgG/AP  碱性磷酸酶（AP）标记的兔抗小鼠IgG

hz-0296R-Bio Rabbit Anti-mouse IgG/Bio  生物素标记的兔抗小鼠IgG

hz-0296R-hzy3 Rabbit Anti-mouse IgG/Cy3  Cy3标记的兔抗小鼠IgG

hz-0296R-hzy5.5 Rabbit Anti-mouse IgG/Cy5.5  Cy5.5标记的兔抗小鼠IgG

hz-0296R-hzy7 Rabbit Anti-mouse IgG/Cy7  Cy7标记的兔抗小鼠IgG

hz-0296R-PE Rabbit Anti-mouse IgG/PE  PE标记的兔抗小鼠IgG

hz-0296R-PE-hzy3 Rabbit Anti-mouse IgG/PE-Cy3  PE-Cy3标记的兔抗小鼠IgG

hz-0296R-PE-hzY5 Rabbit Anti-mouse IgG/PE-CY5  PE-CY5标记的兔抗小鼠IgG

hz-0296R-APhz Rabbit Anti-mouse IgG/APC  APC标记的兔抗小鼠IgG

hz-0296R-AF350 Rabbit Anti-mouse IgG/Alexa Fluor 350  Alexa Fluor 350标记的兔抗小鼠IgG

hz-0296R-AF488 Rabbit Anti-mouse IgG/Alexa Fluor 488  Alexa Fluor 488标记的兔抗小鼠IgG

hz-0296R-AF555 Rabbit Anti-mouse IgG/Alexa Fluor 555  Alexa Fluor 555标记的兔抗小鼠IgG

hz-0296R-AF647 Rabbit Anti-mouse IgG/Alexa Fluor 647  Alexa Fluor 647标记的兔抗小鼠IgG