英文名称 DFNB31
中文名称 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.
说 明 书 0.2ml
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 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 常染色体隐性遗传性耳聋型31蛋白抗体(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 96kDa
细胞定位 细胞核 细胞浆
性 状 Lyophilized or Liquid
浓 度 1mg/1ml
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 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
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.
常染色体隐性遗传性耳聋型31蛋白抗体产品介绍 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.
Gene ID:
25861
常染色体隐性遗传性耳聋型31蛋白抗体
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hz-2179P-hzy3 AACT/Cy3 荧光素Cy3标记胰糜蛋白酶
hz-0294P-A555 Goat IgG/Alexa Fluor 555 Alexa Fluor 555标记羊IgG(细胞流式同型对照)
hz-0293P-A555 Rat IgG/Alexa Fluor 555 Alexa Fluor 555标记大鼠IgG(细胞流式同型对照)
hz-0292P-hzy5 BSA/Cy5 荧光素Cy5标记牛血清白蛋白
hze-0311P Biotin/HRP 辣根过氧化物酶标记生物素
hze-0312P Avidin/HRP 辣根过氧化物酶标记亲合素
hze-0360G Goat Anti-human IgA/HRP 辣根过氧化物酶标记羊抗人IgA
hze-0302R Rabbit Anti-HRP/HRP 辣根过氧化物酶标记兔抗HRP
hze-0360R Rabbit Anti-human IgA/HRP 辣根过氧化物酶标记兔抗人IgA
hze-0437P Streptavidin/HRP 辣根过氧化物酶标记链霉亲和素
hze-0362P Protein A/HRP 辣根过氧化物酶标记的蛋白A
hze-0292P BSA/HRP 辣根过氧化物酶标记牛血清白蛋白
hze-0361P rec Protein G/HRP 辣根过氧化物酶标记的重组蛋白G
hz-0855P-HRP Insulin/HRP 辣根过氧化物酶标记人胰岛素蛋白
hz-0311P-AP Biotin/AP 碱性磷酸酶标记生物素
hz-0312P-AP Avidin/AP 碱性磷酸酶标记亲合素
hz-0437P-AP Streptavidin/AP 碱性磷酸酶标记链霉亲和素
hz-0360G-AP Goat Anti-human IgA/AP 碱性磷酸酶标记羊抗人IgA
hz-0360R-AP Rabbit Anti-human IgA/AP 碱性磷酸酶标记兔抗人IgA
hz-0362P-AP Protein A/AP 碱性磷酸酶标记的蛋白A
hz-0292P-AP BSA/AP 碱性磷酸酶标记标记牛血清白蛋白
hz-0361P-AP rec Protein G/AP 碱性磷酸酶标记的重组蛋白G
hzb-0360G Goat Anti-human IgA/Biotin 生物素化羊抗人IgA
hza-0297R Rabbit Anti-human IgG/Streptavidin 链霉亲和素标记兔抗人IgG
hzb-0295P Rabbit IgG/Biotin 生物素化兔IgG
hzb-0297P human IgG/Biotin 生物素化人IgG
hzb-0296P mouse IgG/Biotin 生物素化小鼠IgG
hzF-0292P BSA/FITC 荧光素标记牛血清白蛋白
hzF-0366R Rb Anti-FITC/FITC 荧光素标记兔抗FITC
hzF-0360G Goat Anti-human IgA/FITC 荧光素标记羊抗人IgA
hzR-0292P BSA/RBITC 罗丹明标记牛血清白蛋白
hzR-0437P Streptavidin/RBITC 红色荧光素罗丹明标记链霉亲和素
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